58:
1291:: The replacement of the bone marrow with bone marrow from an individual without spherocytosis. The transplanted progenitor cells do not have the genetic mutations found in individuals with hereditary spherocytosis, and therefore do not produce spherocytes. This results in an individual with biconcave-disc-shaped erythrocytes. This treatment is not standard of care, and is not offered as it has only been documented incidentally during treatment for other diseases, such as
548:
752:
630:
805:
887:(thickened blood), which can have implications for both larger and smaller diameter vasculature. However, the most pronounced issues with the lack of compliance and fluidity declare themselves in the failure of the erythrocyte to deform itself when transiting arterioles, capillary beds, and venules. These vessels are smaller, and can become congested or blocked altogether. Studies have demonstrated that HS is related to deep vein thrombosis (
683:
1107:: Positive (Spherocytes will rupture in liquid solutions less concentrated than the inside of the red blood cell. This is due to increased permeability of the spherocyte membrane to salt and water, which enters the concentrated inner environment of the RBC and leads to its rupture. The osmotic fragility test is no longer considered the gold standard for diagnosing hereditary spherocytosis, as it misses ~25% of cases).
1249:: This option is non-operative, and is when an interventional radiologist (instead of a surgeon) uses radiologic techniques to coil or cauterize vasculature within the splenic circulation. There are various points in the circulatory pathway where the treatment can be applied to produces varying amounts of retained viability of the spleen, thus giving the option to attempt to preserve splenic immune function.
604:
682:
157:, particularly in newborns. Most cases can be detected soon after birth. Testing for HS is available for the children of affected adults. Occasionally, the disease will go unnoticed until the child is about 4 or 5 years of age. A person may also be a carrier of the disease and show no signs or symptoms of the disease. Late complications may result in the development of
1239:: This is when only a section of the spleen is removed, instead of the entire organ. The goal is to lower the extravascular hemolysis to a level compatible with homeostasis of the patient, while preserving the immune function of the spleen's presence. Research on outcomes is currently ongoing, and is routine at children's hospitals within the United States of America.
804:
1351:(glycated hemoglobin) is a test for determining the average blood glucose levels over an extended period of time, and is often used to evaluate glucose control in diabetics. The hemoglobin A1C levels are abnormally low because the life span of the red blood cells is decreased - providing less time for the non-enzymatic
547:
629:
1338:
Pigmented gallstones occur in approximately half of untreated patients. Increased hemolysis of red blood cells leads to increased bilirubin levels, because bilirubin is a breakdown product of heme. The high levels of bilirubin must be excreted into the bile by the liver, which may cause the formation
273:
trait. The autosomal recessive inheritance pattern accounts for close to 25% of the clinical cases. The autosomal dominant inheritance pattern accounts for over 75% of the clinical cases. Many positive individuals will not present clinically, thus the etiologic data may be artificially skewed towards
1219:
Although research is ongoing, currently there is no genetic-level cure for the myriad of mutations that cause the various presentations of hereditary spherocytosis. Common current management focuses on interventions prevent the body from inappropriately destroying the functional spherocytes produced
898:
The spleen typically acts as a filter for blood, and targets pathogens and other damaged cells within circulation. Removing blood contaminants promotes entire-body homeostasis. The following facts about the spleen's role in normal body functioning are crucial to understanding the implications of HS
929:
In relation to the three facts above, the spleen misguidedly filters spherocytes – regardless of the age or functional status of the cell. The spleen is not dysfunctional itself when selecting spherocytes for destruction, as this is the regular function of the spleen both at an anatomic structure
1142:
Unconjugated bilirubin: Increased (normal range: 0.2–1.2 mg/dL). This is caused by heme released into the hepatosplenic circulation by macrophages that have phagocytosed erythrocytes. The unconjugated bilirubin is not soluble in water (blood), so it binds to albumin, and is processed in the
121:
HS was first described in 1871, and is the most common cause of inherited hemolysis in populations of northern
European descent, with an incidence of 1 in 5000 births. The clinical severity of HS varies from mild (symptom-free carrier), to moderate (anemic, jaundiced, and with splenomegaly), to
886:
Spherocytes have less plasma membrane compliance and fluidity, and this has implications throughout the entirety of circulation within the body, i.e. arteries, arterioles, capillaries, venules, veins, and organs. The lack of compliance and fluidity lead to a higher viscosity value for blood
751:
1130:
Red blood cell count (RBC): Sometimes increased early (normal range males: 4.3–5.9 million/mm; normal range females: 3.5–5.5 million/mm). The loss of surface area per cells cause the body to mass produce red blood cells. The spleen will filter spherocytes out and change this
942:
sample, or "bite", part of the stuck spherocytes' plasma membranes. The macrophages recognize that the spherocyte is not normal, and the macrophage is "programmed" to destroy irregularities filtered at the spleen from the blood. The resident splenic macrophages therefore
1830:
Buesing, K. L.; Tracy, E. T.; Kiernan, C.; Pastor, A. C.; Cassidy, L. D.; Scott, J. P.; Ware, R. E.; Davidoff, A. M.; Rescorla, F. J.; Langer, J. C.; Rice, H. E.; Oldham, K. T. (2011). "Partial splenectomy for hereditary spherocytosis: A multi-institutional review".
1470:
Bolton-Maggs, P. H. B.; Stevens, R. F.; Dodd, N. J.; Lamont, G.; Tittensor, P.; King, M. -J.; General
Haematology Task Force of the British Committee for Standards in Haematology (2004). "Guidelines for the diagnosis and management of hereditary spherocytosis".
1267:. Antibiotics are no longer recommended for maintenance use, even in post-splenectomy HS patients. Since the spleen is important for protecting against encapsulated organisms, sepsis caused by encapsulated organisms is a possible complication of splenectomy.
1191:
may cause additional health issues. Measuring iron stores is sometimes considered part of the diagnostic approach to hereditary spherocytosis in older patients presenting with heart muscle damage of unknown etiology or liver disease without apparent cause.
1900:
1885:
205:
levels. The resultant asplenic patient is susceptible to encapsulated bacterial infection, and prevented with vaccination. If other symptoms, such as abdominal pain persist, the removal of the gallbladder may be warranted for symptomatic
494:
which is responsible for incorporation and binding of spectrin to the greater actin cytoskeleton. This dysfunction of cytoskeletal instabilities ensue, and leave the plasma membrane of the cell less supported and/or weakened.
224:, the frequency is at least 1 in 5,000 within the United States of America. While HS is most commonly (though not exclusively) found in Northern European and Japanese families, an estimated 25% of cases are due to spontaneous
993:(with secondary pallor), and a palpable spleen sometimes with concomitant tenderness (due to splenic congestion and splenomegaly). Its worth noting that a subsection of HS patients will also have incidental black pigmented
274:
the more prominent dominant forms. These dominant forms tend to leave a family history that yields generational splenectomies and black gallstones cholelithiasis. Lastly, an estimated 25% of cases are due to spontaneous
1134:
Reticulocytes: Increased (normal range: 0.5–1.5% of the RBC listed above). The body to mass produces red blood cells (reticulocytes being young erythrocytes) even as the spleen filters spherocytes out. This is known as
3145:
1339:
of a pigmented gallstone, which is composed of calcium bilirubinate. Since these stones contain high levels of calcium carbonates and phosphate, they are radiopaque and are visible on x-ray.
1233:: This is the operative removal of the entire spleen by a surgeon, thus stopping the extravascular hemolysis, but also removing the immune functions the spleen naturally provide to the body.
1163:: Positive (reduced mean fluorescence), as the test will demonstrate a reduced ability of the eosin-5-maleimide dye to bind to erythrocyte plasma membrane proteins. The process relies upon
3419:
1127:
Red blood cell distribution width (RDW): Increased (normal range: 11–15%). The spherocytes create variation in the size of the red blood cells on average, thus expanding the distribution.
1113:: Positive (A newer version of the osmotic fragility test that adds glycerol to a hypotonic solution. This produces lysis, a positive test, quicker that the traditional saline version).
874:
Defects of band 3 lead to band 3 deficiency and loss of its lipid-stabilizing effect within the plasma membrane lipid bilayer. This results in the release of band 3-free microvesicles.
855:
A secondary defect in hereditary spherocytosis is a deficiency of membrane surface area. The decrease in surface area leads to less efficient gas exchange of the erythrocyte at the
863:
Defects of spectrin, ankyrin (most commonly), or PROTEIN 4.2 lead to reduced structural integrity of the plasma membrane, destabilizing the overlying lipid bilayer, and releasing
3381:
878:
Both pathways result in compromised plasma membrane integrity, decreased surface area, and formation of spherocytes with decreased mechanical compliance during circulation.
2781:
106:), rather than the normal biconcave disk shape. This abnormal shape interferes with the cells' ability to flex during blood circulation, and also makes them more prone to
2978:
1386:
exists to treat hereditary spherocytosis in lab mice; however, this treatment has not yet been tried on humans due to all of the risks involved in human gene therapy.
490:
These proteins are necessary to maintain the normal shape of a red blood cell, which is a biconcave disk. The integrating protein that is most commonly defective is
30:
This article is about the hereditary hematologic manifestation of spherocytic disorders. For details that pertain to the mechanical state of spherocytic cells, see
934:
into the sinusoids may be seen as a "bottleneck", where red blood cells need to be flexible in order to pass through. In HS, the erythrocytes fail to pass through
2914:
2864:
2558:
967:
HS patients present in a vast array of presentations, from being asymptomatic to the extreme situations of splenic rupture, hemolytic crisis, or in-utero demise.
220:
2165:
1121:
Mean cell volume (MCV): Normocytic (normal range: 80–100 fL), or slightly lower. Spherocytes are slightly smaller than normal biconcave red blood cells.
2983:
2370:
1324:
Aplastic crisis with dramatic fall in hemoglobin level and (reticulocyte count)-decompensation, usually due to maturation arrest and often associated with
122:
severe (hemolytic crisis, in-utero hydrops fetalis), because HS is caused by genetic mutations in a multitude of structural membrane proteins and exhibits
3402:
3370:
2521:
2023:
2473:
2295:
1146:
Haptoglobin (free): Decreased (normal range: 41–165 mg/dL). This is caused by hemoglobin binding to haptoglobin, thus making it no longer "free".
1036:
3105:
2365:
3095:
2305:
3087:
1286:
800:
Reduced density of plasma cell membrane skeleton, destabilizing the plasma lipid bilayer, thus releasing band-3 protein-containing microvesicles.
1541:
1124:
Mean corpuscular hemoglobin concentration (MCHC): Increased (normal range: 31–36% Hb/cell). This is secondary to less water being in the cell.
3463:
3453:
3386:
1761:
3178:
3055:
3003:
2182:
710:
to interior plasma membrane, and subsequently weakened association between the greater actin cytoskeleton and the plasma membrane (bottom).
696:
to interior plasma membrane, and subsequently weakened association between the greater actin cytoskeleton and the plasma membrane (bottom).
2337:
1915:
467:
236:
Hereditary spherocytosis is an erythrocytic disorder of that affects the following red cell membrane proteins in a congenital fashion:
2551:
1717:
Eber S, Lux SE (April 2004). "Hereditary spherocytosis--defects in proteins that connect the membrane skeleton to the lipid bilayer".
1607:
218:
Hereditary spherocytosis is the heritable hemolytic disorder, affecting 1 in 2,000 people of
Northern European ancestry. According to
3211:
2346:
2317:
1447:
1040:
2249:
1515:
3206:
2377:
1596:
Anthony S. Fauci; Eugene
Braunwald; Dennis L. Kasper; Stephen L. Hauser; Dan L. Longo; J. Larry Jameson; Joseph Loscalzo (2008).
3125:
3015:
177:
or other illness can cause an increase in the destruction of red blood cells, resulting in the appearance of acute symptoms, a
3433:
3314:
3268:
3100:
3090:
2310:
2016:
836:
of the greater actin cytoskeleton to associate with the plasma membrane via spectrin and ankyrin, and the subsequent loss of
818:
of the greater actin cytoskeleton to associate with the plasma membrane via spectrin and ankyrin, and the subsequent loss of
719:
251:
3115:
2754:
2356:
1149:
Lactate dehydrogenase (LDH): Increased (normal range: 110–295 U/L in children). This is due to extravascular hemolysis.
470:). The Alpha-1 refers the alpha-1 subunit of the spectrin protein. The Beta refers the beta subunit of the spectrin protein.
291:
Hereditary spherocytosis is caused by a variety of molecular defects in the genes that code for the red blood cell proteins
3458:
3263:
3232:
3183:
3060:
3030:
2934:
2884:
2854:
2544:
2404:
1203:
is often used to evaluate the dimensions of the spleen, and also the gallbladder in preparation for functionally curative
2650:
1516:"Complete nucleotide sequence of insertion element IS4351 from Bacteroides fragilis. - UKPMC Article - UK PubMed Central"
2904:
2838:
2261:
956:
479:
2413:
2106:
2724:
2701:
2678:
2640:
2602:
2287:
2151:
1991:
1098:
955:
and anemia. Should this process continue unchecked chronically, inappropriate regulation of erythropoeisis leads to
3303:
3284:
3201:
2351:
2177:
997:(made of calcium bilirubinate consequence of the extravascular hemolysis), and some of these patients will develop
2278:
2268:
2256:
2009:
1321:
Hemolytic crisis, with more pronounced jaundice due to accelerated hemolysis (may be precipitated by infection).
57:
3365:
3360:
3258:
3082:
2833:
1926:
1292:
1259:
1244:
619:
3140:
2660:
859:
of the lungs and throughout circulation. Decreased surface area may be produced by two different mechanisms:
478:
cells within the bone marrow, where red blood cells are normally produced in the non-pathological state (see
3397:
3339:
2944:
2449:
2300:
948:
475:
110:
under osmotic stress, mechanical stress, or both. Cells with the dysfunctional proteins are degraded in the
173:
gene may exhibit iron overload, despite the hemochromatosis genes being recessive. In chronic patients, an
3050:
3008:
2607:
2440:
2101:
1438:
Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005).
1069:
1052:
1026:
986:
892:
794:
2797:
2655:
2612:
2420:
2187:
1977:
1060:
127:
123:
3309:
1548:
189:
may be seen within red blood cells. Primary treatment for patients with symptomatic HS has been total
2430:
2425:
2233:
2119:
1325:
1168:
1044:
888:
158:
2771:
2479:
2224:
2032:
1904:
1573:
1395:
Evolution of spherocytic erythrocyte adaptation in those of
Northern European and Japanese descent.
1006:
935:
779:
without integral band-3 protein for association with protein-4.2 and subsequently ankyrin (bottom).
765:
without integral band-3 PROTEIN for association with protein-4.2 and subsequently ANKYRIN (bottom).
266:
186:
3278:
2954:
2924:
2894:
2874:
2843:
2511:
2484:
2089:
1803:
1699:
1496:
856:
270:
1630:"Hematologically important mutations: spectrin and ankyrin variants in hereditary spherocytosis"
1253:
All operative and interventional treatments require the immunization of HS patients against the
1996:
3019:
2706:
2683:
2635:
2617:
2516:
2506:
2465:
2460:
2455:
2396:
2228:
2217:
2207:
2141:
2124:
2115:
2097:
2059:
1955:
1848:
1795:
1757:
1734:
1691:
1651:
1603:
1488:
1443:
1184:
931:
530:
404:
161:, which is secondary to the detritus of the broken-down blood cells (unconjugated or indirect
142:
138:
70:
1519:
1355:
of hemoglobin. Thus, even with high overall blood sugar, the A1C will be lower than expected.
3246:
2212:
2197:
2136:
1840:
1787:
1726:
1683:
1641:
1480:
1032:
725:
603:
115:
91:
17:
1778:
Won DI, Suh JS (March 2009). "Flow cytometric detection of erythrocyte osmotic fragility".
3130:
2812:
2776:
2749:
2391:
1398:
Increased ability to defend against viral infections in hereditary spherocytosis patients.
1274:
1208:
1180:
1136:
1090:
The common findings of lab testing in setting of a patient with hereditary spherocytosis:
741:
170:
99:
1597:
930:
level, and at the molecular cellular level. At the anatomic level, the passage from the
3327:
2824:
2645:
2408:
2331:
2239:
2036:
1752:
Mitchell, Richard
Sheppard; Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson (2007). "12".
1730:
1348:
1329:
1164:
998:
922:
664:
of
Spectrin interior to the plasma membrane to the greater actin cytoskeleton (bottom).
647:
of spectrin interior to the plasma membrane to the greater actin cytoskeleton (bottom).
575:
of
Spectrin interior to the plasma membrane to the greater actin cytoskeleton (bottom).
561:
of spectrin interior to the plasma membrane to the greater actin cytoskeleton (bottom).
1909:
1687:
706:
that normally associates with plasma membrane proteins (top). The resulting failed or
692:
that normally associates with plasma membrane proteins (top). The resulting failed or
3447:
3046:
2576:
1484:
1408:
1352:
1342:
1264:
1188:
1002:
868:
103:
31:
1703:
2567:
2051:
1966:
1844:
1807:
1500:
1383:
1048:
952:
944:
540:
198:
134:
921:
Removal of damaged erythrocytes continues healthy signaling to bone marrow, where
1931:
1674:
Perrotta S, Gallagher PG, Mohandas N (October 2008). "Hereditary spherocytosis".
1392:
Paradoxical endurance-based athleticism associated with hereditary spherocytosis.
918:
via hepatosplenic recycling, which reduces sepsis risk (see
Complications below).
3393:
3356:
3322:
3161:
3076:
2972:
2820:
2766:
2693:
2585:
2202:
2160:
1950:
1228:
1204:
1078:
1056:
788:
733:
312:
256:
190:
166:
150:
678:
Failure to mediate anchorage of integral plasma membrane proteins to spectrin.
3409:
3026:
2950:
2940:
2930:
2920:
2910:
2900:
2850:
2171:
2001:
1894:
1418:
1200:
939:
194:
182:
75:
3334:
3227:
2890:
2880:
2870:
2860:
2828:
2816:
2744:
1961:
1254:
994:
207:
202:
174:
162:
154:
107:
95:
1852:
1799:
1738:
1695:
1646:
1629:
1492:
1328:
changes; may be precipitated by infection, such as influenza, notably with
1655:
3254:
2670:
1972:
1791:
1542:"Guidelines for the Diagnosis and Management of Hereditary Spherocytosis"
1022:
Available lab testing that may aid in the diagnosis of HS is as follows:
982:
729:
614:
526:
491:
292:
275:
240:
225:
3377:
3294:
3274:
3170:
2761:
2536:
670:
304:
246:
1877:
1602:(17th ed.). New York: McGraw-Hill Medical. pp. Chapter 106.
803:
750:
747:
Deficiency causes reduced lipid-stabilization at the plasma membrane.
681:
675:
Hydrophobic interactions and electrostatic attraction to Beta subunit
628:
546:
3193:
2999:
2627:
2080:
1889:
1413:
1303:
990:
904:
864:
427:
308:
146:
111:
1547:. The British Committee for Standards in Haematology. Archived from
903:
Removal of pathogens includes encapsulated bacteria, which reduces
2968:
2964:
2960:
2716:
2594:
602:
450:
296:
65:
Peripheral blood smear from patient with hereditary spherocytosis
3414:
1920:
1176:
915:
444:
421:
398:
381:
375:
358:
352:
332:
300:
2540:
2005:
797:
binding that regulates the association of band-3 with ankyrin.
1335:
Folate deficiency caused by increased bone marrow requirement.
625:
Failure in plasma membrane tethering to actin cytoskeleton.
3146:
Hydrops-ectopic calcification-moth-eaten skeletal dysplasia
977:
Neonatal or in-utero onset HS (severe): <5% of patients.
114:, which leads to a shortage of erythrocytes and results in
1001:
or the potential complex sequelae of this condition, i.e.
657:
subunits in spectrin protein network (top). The resulting
639:
subunits in spectrin protein network (top). The resulting
571:
subunits in spectrin protein network (top). The resulting
557:
subunits in spectrin protein network (top). The resulting
193:, which eliminates the hemolytic process, allowing normal
3420:
Microcephalic osteodysplastic primordial dwarfism type II
914:
Removal of damaged erythrocytes reduces iron burden from
486:
Pathophysiology of mutated erythrocytic membrane proteins
482:
for pathological production outside of the bone marrow).
1220:
by erythrocyte progenitor cells within the bone marrow.
27:
Genetic disorder causing red blood cells to be spherical
1171:
test that produces results at low cost within ~2 hours.
871:. Band-3 is important for gas exchange (as seen above).
474:
These genetic mutations are acted upon and executed by
90:) is a congenital hemolytic disorder wherein a genetic
1514:
J L Rasmussen; D A Odelson; F L Macrina (1987-08-01).
287:
Causative genetic mutations and phenotypic expressions
3382:
Epidermolysis bullosa simplex with muscular dystrophy
1465:
1463:
1461:
1459:
1257:
virus, SARS-CoV-2, and encapsulated bacteria such as
1867:
3349:
3293:
3245:
3220:
3192:
3169:
3160:
3069:
3039:
2992:
2805:
2796:
2737:
2715:
2692:
2669:
2626:
2593:
2584:
2575:
2499:
2439:
2390:
2286:
2277:
2150:
2135:
2088:
2079:
2072:
2050:
2043:
1941:
1871:
1591:
1589:
1587:
1585:
1583:
69:
47:
42:
2782:Terminal osseous dysplasia with pigmentary defects
974:Infantile-onset HS (moderate): 60–75% of patients.
622:and electrostatic attraction to ankyrin and actin
1442:. St. Louis, Mo: Elsevier Saunders. p. 625.
1574:"Hereditary spherocytosis: MedlinePlus Genetics"
1820:Goljan. Rapid Review Pathology. 2010. Page 213.
925:replaces red blood cells lost to wear and tear.
2915:Meesmann juvenile epithelial corneal dystrophy
2865:Meesmann juvenile epithelial corneal dystrophy
1669:
1667:
1665:
1440:Robbins and Cotran pathologic basis of disease
981:The most common presentation will demonstrate
539:Failure in plasma membrane tethering to actin
315:, and other red blood cell membrane proteins:
2552:
2017:
1623:
1621:
1619:
8:
3403:Arrhythmogenic right ventricular dysplasia 9
3371:Arrhythmogenic right ventricular dysplasia 8
2166:Glucose-6-phosphate dehydrogenase deficiency
1345:with concomitant low hemoglobin A1C levels.
169:. Also, patients who are heterozygous for a
2984:Reticular pigmented anomaly of the flexures
1157:: Directly shows spherocytes on microscope.
971:Asymptomatic HS (mild): 20–30% of patients.
3166:
2802:
2590:
2581:
2559:
2545:
2537:
2522:Hereditary persistence of fetal hemoglobin
2283:
2147:
2085:
2076:
2047:
2024:
2010:
2002:
1868:
1599:Harrison's principles of internal medicine
1175:In chronic cases, patients who have taken
221:Harrison's Principles of Internal Medicine
56:
39:
2474:Mean corpuscular hemoglobin concentration
2296:Warm antibody autoimmune hemolytic anemia
1645:
1628:Gallagher PG, Forget BG (December 1998).
1270:Additional elective treatments offered:
1037:mean corpuscular hemoglobin concentration
899:on the overall health of the individual:
133:Early symptoms include anemia, jaundice,
94:coding for a structural membrane protein
2979:Naegeli–Franceschetti–Jadassohn syndrome
497:
317:
1756:. Philadelphia: Saunders. p. 425.
1430:
466:*Online Mendelian Inheritance in Man (
344:Erythrocyte membrane protein Affected
3387:Epidermolysis bullosa simplex of Ogna
2656:DFN A3, 4, 11, 17, 22; B2, 30, 37, 48
1997:A picture of spherocytes from Medline
1540:Paula Bolton-Maggs (September 2011).
938:, and this is where resident splenic
593:Decreased plasma membrane compliance.
7:
3004:Desmin-related myofibrillar myopathy
2641:Hypertrophic cardiomyopathy 1, 8, 10
2183:triosephosphate isomerase deficiency
607:Micrograph of a spherocyte (center).
141:. Acute cases can threaten to cause
3096:Emery–Dreifuss muscular dystrophy 2
2338:paroxysmal nocturnal hemoglobinuria
2306:Donath–Landsteiner hemolytic anemia
1378:Modern ongoing research interests:
1275:Surgical removal of the gallbladder
1223:Typical treatment options include:
1179:supplementation, have heterozygous
1077:A negative direct antiglobin test (
838:Band-3 Protein within microvesicles
820:band-3 protein within microvesicles
265:Hereditary spherocytosis can be an
3361:Striate palmoplantar keratoderma 2
3056:Charcot–Marie–Tooth disease 1F, 2E
2834:Striate palmoplantar keratoderma 3
1731:10.1053/j.seminhematol.2004.01.002
908:
584:Erythrocytic plasma membrane loss.
536:Alpha-1 subunit actin association
25:
3212:Asphyxiating thoracic dysplasia 3
3101:Limb-girdle muscular dystrophy 1B
2347:Microangiopathic hemolytic anemia
2318:Mixed autoimmune hemolytic anemia
1041:red blood cell distribution width
882:Cardiovascular and organ sequelae
3207:Short rib-polydactyly syndrome 3
3184:Hereditary spastic paraplegia 10
2679:Hypertrophic cardiomyopathy 7, 2
2378:Hemolytic disease of the newborn
1485:10.1111/j.1365-2141.2004.05052.x
3106:Charcot–Marie–Tooth disease 2B1
1518:. UKPMC Article. Archived from
1237:Partial splenectomy (operative)
261:Lesser proteins of significance
3315:Familial adenomatous polyposis
3269:Hereditary elliptocytosis 2, 3
3179:Charcot–Marie–Tooth disease 2A
3091:Familial partial lipodystrophy
2603:Hypertrophic cardiomyopathy 11
2311:Paroxysmal cold hemoglobinuria
1845:10.1016/j.jpedsurg.2010.09.090
1473:British Journal of Haematology
1161:Eosin-5-maleimide binding test
1066:Eosin-5-maleimide binding test
708:weakened anchorage of spectrin
321:Hereditary Spherocytosis Type
1:
3264:Hereditary spherocytosis 2, 3
3233:Cavernous venous malformation
3061:Amyotrophic lateral sclerosis
3031:Amyotrophic lateral sclerosis
2935:Epidermolysis bullosa simplex
2885:Epidermolysis bullosa simplex
2855:Ichthyosis bullosa of Siemens
2725:Hypertrophic cardiomyopathy 9
2702:Hypertrophic cardiomyopathy 3
1688:10.1016/S0140-6736(08)61588-3
1111:Acidified glycerol lysis test
1074:Acidified glycerol lysis test
694:weakened anchorage of spectin
506:Protein Function Compromised
503:Erythrocyte Membrane Protein
153:through high blood levels of
3464:Autosomal dominant disorders
3454:Hereditary hemolytic anemias
2905:Epidermolytic hyperkeratosis
2839:Epidermolytic hyperkeratosis
2262:Southeast Asian ovalocytosis
2250:Minkowski–Chauffard syndrome
1833:Journal of Pediatric Surgery
1289:(heme-oncology intervention)
957:extramedullary hematopoiesis
777:destabilized plasma membrane
763:destabilized plasma membrane
573:failed or weakened tethering
559:failed or weakened tethering
480:Extramedullary hematopoiesis
51:Minkowski–Chauffard syndrome
18:Minkowski–Chauffard syndrome
3126:Buschke–Ollendorff syndrome
1366:Deep vein thrombosis (DVT).
1099:autoimmune hemolytic anemia
3480:
3285:Hereditary spherocytosis 1
3202:Primary ciliary dyskinesia
2608:Dilated cardiomyopathy 1AA
2371:Drug-induced nonautoimmune
2352:Thrombotic microangiopathy
2178:pyruvate kinase deficiency
1992:A short article from WebMD
1086:Common laboratory findings
165:) accumulating within the
29:
3429:
3116:Barraquer–Simons syndrome
3009:Dilated cardiomyopathy 1I
2755:Weill–Marchesani syndrome
2357:Hemolytic–uremic syndrome
2269:Hereditary stomatocytosis
2257:Hereditary elliptocytosis
947:the spherocytes, causing
587:Formation of spherocytes.
581:
578:
323:
320:
64:
55:
3259:Spinocerebellar ataxia 5
3083:Mandibuloacral dysplasia
2651:Freeman–Sheldon syndrome
2245:Hereditary spherocytosis
1299:Symptomatic treatments:
1293:myelodysplastic syndrome
1281:Experimental treatment:
1260:Streptococcus pneumoniae
1245:interventional radiology
620:Hydrophobic interactions
84:Hereditary spherocytosis
43:Hereditary spherocytosis
3398:Skin fragility syndrome
3340:Giant axonal neuropathy
2945:Steatocystoma multiplex
2450:Mean corpuscular volume
2414:Diamond–Blackfan anemia
2366:Drug-induced autoimmune
2301:Cold agglutinin disease
2107:Plummer–Vinson syndrome
1754:Robbins Basic Pathology
1389:Bone marrow transplant.
1369:Cardiovascular disease.
1183:, or received numerous
1031:Supportive blood work:
949:extravascular hemolysis
724:Failure to mediate the
590:Decreased surface area.
2684:Nemaline myopathy 4, 5
2102:Iron-deficiency anemia
1647:10.1006/bcmd.1998.0217
1287:Bone marrow transplant
1155:Peripheral blood smear
1117:Supportive blood work:
1105:Osmotic fragility test
1097:: Negative (rules-out
1070:Osmotic fragility test
1053:unconjugated bilirubin
1027:Peripheral blood smear
987:unconjugated bilirubin
893:cardiovascular disease
824:
767:
698:
649:
608:
563:
476:erythrocyte progenitor
327:Phenotypic Expression
3434:Cytoskeletal proteins
2421:Pure red cell aplasia
2188:hexokinase deficiency
1061:lactate dehydrogenase
963:Clinical presentation
951:. This leads to both
851:Cellular implications
832:(top). The resulting
814:(top). The resulting
807:
775:(top). The resulting
761:(top). The resulting
754:
685:
632:
606:
550:
124:incomplete penetrance
102:to be sphere-shaped (
3459:Cytoskeletal defects
2426:Sideroblastic anemia
2234:Hemoglobin C disease
2120:Megaloblastic anemia
1792:10.1002/cyto.b.20448
1634:Blood Cells Mol. Dis
1316:Common complications
1045:red blood cell count
413:Spectrin (Alpha-1)*
159:pigmented gallstones
3141:Pelger–Huet anomaly
3051:Parkinson's disease
2772:Boomerang dysplasia
2707:Nemaline myopathy 1
2661:May–Hegglin anomaly
2618:Nemaline myopathy 3
2225:Sickle cell disease
1007:choledocholithiasis
512:Pathogenesis of HS
499:
324:Genotypic Etiology
267:autosomal recessive
187:Howell-Jolly bodies
3310:Gardner's syndrome
3279:Long QT syndrome 4
2955:Familial cirrhosis
2925:White sponge nevus
2895:Familial cirrhosis
2875:White sponge nevus
2512:Sulfhemoglobinemia
1942:External resources
1243:Splenic ablation (
1185:blood transfusions
1018:Laboratory testing
985:(due to increased
828:The deficiency of
825:
810:The deficiency of
771:The deficiency of
768:
757:The deficiency of
702:The deficiency of
699:
688:The deficiency of
662:weakened tethering
653:The deficiency of
650:
645:weakened tethering
635:The deficiency of
609:
567:The deficiency of
564:
553:The deficiency of
518:Mechanical Effect
498:
271:autosomal dominant
3441:
3440:
3366:Carvajal syndrome
3241:
3240:
3156:
3155:
3020:Alexander disease
2792:
2791:
2733:
2732:
2646:Usher syndrome 1B
2636:Elejalde syndrome
2534:
2533:
2530:
2529:
2517:Reticulocytopenia
2507:Methemoglobinemia
2495:
2494:
2386:
2385:
2326:
2325:
2125:Pernicious anemia
2068:
2067:
2060:Polycythemia vera
1987:
1986:
1763:978-1-4160-2973-1
1682:(9647): 1411–26.
932:cords of Billroth
895:for this reason.
848:
847:
463:
462:
390:Spectrin (Beta)*
81:
80:
37:Medical condition
16:(Redirected from
3471:
3432:Related topics:
3167:
2803:
2591:
2582:
2561:
2554:
2547:
2538:
2284:
2198:hemoglobinopathy
2148:
2086:
2077:
2048:
2026:
2019:
2012:
2003:
1869:
1857:
1856:
1827:
1821:
1818:
1812:
1811:
1780:Cytometry Part B
1775:
1769:
1767:
1749:
1743:
1742:
1714:
1708:
1707:
1671:
1660:
1659:
1649:
1625:
1614:
1613:
1593:
1578:
1577:
1570:
1564:
1563:
1561:
1559:
1553:
1546:
1537:
1531:
1530:
1528:
1527:
1511:
1505:
1504:
1467:
1454:
1453:
1435:
1306:supplementation.
1207:with or without
1033:mean cell volume
742:plasma membranes
500:
318:
243:(alpha and beta)
179:hemolytic crisis
116:hemolytic anemia
60:
40:
21:
3479:
3478:
3474:
3473:
3472:
3470:
3469:
3468:
3444:
3443:
3442:
3437:
3425:
3345:
3289:
3237:
3216:
3188:
3152:
3131:Osteopoikilosis
3065:
3035:
2988:
2788:
2777:Larsen syndrome
2750:Marfan syndrome
2729:
2711:
2688:
2665:
2622:
2571:
2565:
2535:
2526:
2491:
2435:
2394:
2382:
2322:
2273:
2139:
2131:
2064:
2039:
2037:red blood cells
2030:
1988:
1983:
1982:
1937:
1936:
1880:
1866:
1861:
1860:
1829:
1828:
1824:
1819:
1815:
1777:
1776:
1772:
1764:
1751:
1750:
1746:
1716:
1715:
1711:
1673:
1672:
1663:
1627:
1626:
1617:
1610:
1595:
1594:
1581:
1572:
1571:
1567:
1557:
1555:
1551:
1544:
1539:
1538:
1534:
1525:
1523:
1513:
1512:
1508:
1469:
1468:
1457:
1450:
1437:
1436:
1432:
1427:
1405:
1376:
1318:
1313:
1217:
1209:cholecystectomy
1198:
1181:hemochromatosis
1137:reticulocytosis
1088:
1020:
1015:
965:
909:§ Complications
891:) and arterial
884:
853:
739:
615:Spectrin (Beta)
509:Process Effect
488:
436:Band-3 Protein
289:
284:
282:Pathophysiology
234:
216:
171:hemochromatosis
100:red blood cells
38:
35:
28:
23:
22:
15:
12:
11:
5:
3477:
3475:
3467:
3466:
3461:
3456:
3446:
3445:
3439:
3438:
3430:
3427:
3426:
3424:
3423:
3406:
3405:
3400:
3390:
3389:
3384:
3374:
3373:
3368:
3363:
3353:
3351:
3347:
3346:
3344:
3343:
3331:
3328:Naxos syndrome
3319:
3318:
3317:
3312:
3299:
3297:
3291:
3290:
3288:
3287:
3272:
3271:
3266:
3261:
3251:
3249:
3243:
3242:
3239:
3238:
3236:
3235:
3230:
3224:
3222:
3218:
3217:
3215:
3214:
3209:
3204:
3198:
3196:
3190:
3189:
3187:
3186:
3181:
3175:
3173:
3164:
3158:
3157:
3154:
3153:
3151:
3150:
3149:
3148:
3143:
3135:
3134:
3133:
3128:
3120:
3119:
3118:
3110:
3109:
3108:
3103:
3098:
3093:
3085:
3073:
3071:
3067:
3066:
3064:
3063:
3058:
3053:
3043:
3041:
3037:
3036:
3034:
3033:
3023:
3022:
3012:
3011:
3006:
2996:
2994:
2990:
2989:
2987:
2986:
2981:
2976:
2958:
2948:
2938:
2928:
2918:
2908:
2898:
2888:
2878:
2868:
2858:
2848:
2847:
2846:
2841:
2836:
2825:hyperkeratosis
2809:
2807:
2800:
2794:
2793:
2790:
2789:
2787:
2786:
2785:
2784:
2779:
2774:
2769:
2759:
2758:
2757:
2752:
2741:
2739:
2735:
2734:
2731:
2730:
2728:
2727:
2721:
2719:
2713:
2712:
2710:
2709:
2704:
2698:
2696:
2690:
2689:
2687:
2686:
2681:
2675:
2673:
2667:
2666:
2664:
2663:
2658:
2653:
2648:
2643:
2638:
2632:
2630:
2624:
2623:
2621:
2620:
2615:
2610:
2605:
2599:
2597:
2588:
2579:
2577:Microfilaments
2573:
2572:
2566:
2564:
2563:
2556:
2549:
2541:
2532:
2531:
2528:
2527:
2525:
2524:
2519:
2514:
2509:
2503:
2501:
2497:
2496:
2493:
2492:
2490:
2489:
2488:
2487:
2482:
2470:
2469:
2468:
2463:
2458:
2445:
2443:
2437:
2436:
2434:
2433:
2428:
2423:
2416:
2411:
2409:Fanconi anemia
2402:
2400:
2388:
2387:
2384:
2383:
2381:
2380:
2374:
2373:
2368:
2362:
2361:
2360:
2359:
2349:
2343:
2342:
2341:
2340:
2327:
2324:
2323:
2321:
2320:
2315:
2314:
2313:
2303:
2298:
2292:
2290:
2281:
2275:
2274:
2272:
2271:
2266:
2265:
2264:
2254:
2253:
2252:
2236:
2231:
2222:
2221:
2220:
2215:
2210:
2193:
2192:
2191:
2190:
2185:
2180:
2168:
2156:
2154:
2145:
2133:
2132:
2130:
2129:
2128:
2127:
2112:
2111:
2110:
2109:
2094:
2092:
2083:
2074:
2070:
2069:
2066:
2065:
2063:
2062:
2056:
2054:
2045:
2041:
2040:
2031:
2029:
2028:
2021:
2014:
2006:
2000:
1999:
1994:
1985:
1984:
1981:
1980:
1969:
1958:
1946:
1945:
1943:
1939:
1938:
1935:
1934:
1923:
1912:
1897:
1881:
1876:
1875:
1873:
1872:Classification
1865:
1864:External links
1862:
1859:
1858:
1839:(1): 178–183.
1822:
1813:
1770:
1762:
1744:
1719:Semin. Hematol
1709:
1661:
1615:
1609:978-0071466332
1608:
1579:
1565:
1554:on 12 May 2012
1532:
1506:
1479:(4): 455–474.
1455:
1448:
1429:
1428:
1426:
1423:
1422:
1421:
1416:
1411:
1404:
1401:
1400:
1399:
1396:
1393:
1390:
1387:
1375:
1372:
1371:
1370:
1367:
1364:
1361:
1360:Iron overload.
1358:
1357:
1356:
1349:Hemoglobin A1C
1340:
1336:
1333:
1330:parvovirus B19
1322:
1317:
1314:
1312:
1309:
1308:
1307:
1297:
1296:
1279:
1278:
1251:
1250:
1240:
1234:
1216:
1213:
1197:
1194:
1173:
1172:
1165:flow cytometry
1158:
1152:
1151:
1150:
1147:
1144:
1140:
1132:
1128:
1125:
1122:
1114:
1108:
1102:
1087:
1084:
1083:
1082:
1075:
1072:
1067:
1064:
1029:
1019:
1016:
1014:
1011:
999:cholelithiasis
979:
978:
975:
972:
964:
961:
927:
926:
923:erythropoiesis
919:
912:
883:
880:
876:
875:
872:
852:
849:
846:
845:
842:
801:
798:
791:
785:
784:
781:
773:band-3 protein
759:band-3 protein
748:
745:
737:
722:
716:
715:
712:
679:
676:
673:
667:
666:
626:
623:
617:
611:
610:
601:
600:
597:
594:
591:
588:
585:
580:
577:
544:
537:
534:
523:
522:
516:
513:
510:
507:
504:
487:
484:
461:
460:
457:
454:
447:
442:
438:
437:
434:
431:
424:
419:
415:
414:
411:
408:
401:
396:
392:
391:
388:
385:
378:
373:
369:
368:
365:
362:
355:
350:
346:
345:
342:
339:
336:
329:
328:
325:
322:
288:
285:
283:
280:
263:
262:
259:
254:
252:Band-3 protein
249:
244:
233:
230:
215:
212:
208:cholelithiasis
79:
78:
73:
67:
66:
62:
61:
53:
52:
49:
45:
44:
36:
26:
24:
14:
13:
10:
9:
6:
4:
3:
2:
3476:
3465:
3462:
3460:
3457:
3455:
3452:
3451:
3449:
3436:
3435:
3428:
3421:
3417:
3416:
3411:
3408:
3407:
3404:
3401:
3399:
3395:
3392:
3391:
3388:
3385:
3383:
3379:
3376:
3375:
3372:
3369:
3367:
3364:
3362:
3358:
3355:
3354:
3352:
3348:
3341:
3337:
3336:
3332:
3329:
3325:
3324:
3320:
3316:
3313:
3311:
3308:
3307:
3306:
3305:
3301:
3300:
3298:
3296:
3292:
3286:
3283:
3282:
3281:
3280:
3276:
3270:
3267:
3265:
3262:
3260:
3256:
3253:
3252:
3250:
3248:
3244:
3234:
3231:
3229:
3226:
3225:
3223:
3219:
3213:
3210:
3208:
3205:
3203:
3200:
3199:
3197:
3195:
3191:
3185:
3182:
3180:
3177:
3176:
3174:
3172:
3168:
3165:
3163:
3159:
3147:
3144:
3142:
3139:
3138:
3136:
3132:
3129:
3127:
3124:
3123:
3121:
3117:
3114:
3113:
3111:
3107:
3104:
3102:
3099:
3097:
3094:
3092:
3089:
3086:
3084:
3081:
3080:
3078:
3075:
3074:
3072:
3068:
3062:
3059:
3057:
3054:
3052:
3048:
3047:Neurofilament
3045:
3044:
3042:
3038:
3032:
3028:
3025:
3024:
3021:
3017:
3014:
3013:
3010:
3007:
3005:
3001:
2998:
2997:
2995:
2991:
2985:
2982:
2980:
2977:
2974:
2970:
2966:
2962:
2959:
2956:
2952:
2949:
2946:
2942:
2939:
2936:
2932:
2929:
2926:
2922:
2919:
2916:
2912:
2909:
2906:
2902:
2899:
2896:
2892:
2889:
2886:
2882:
2879:
2876:
2872:
2869:
2866:
2862:
2859:
2856:
2852:
2849:
2845:
2842:
2840:
2837:
2835:
2832:
2831:
2830:
2826:
2822:
2818:
2814:
2813:Keratinopathy
2811:
2810:
2808:
2804:
2801:
2799:
2795:
2783:
2780:
2778:
2775:
2773:
2770:
2768:
2767:FG syndrome 2
2765:
2764:
2763:
2760:
2756:
2753:
2751:
2748:
2747:
2746:
2743:
2742:
2740:
2736:
2726:
2723:
2722:
2720:
2718:
2714:
2708:
2705:
2703:
2700:
2699:
2697:
2695:
2691:
2685:
2682:
2680:
2677:
2676:
2674:
2672:
2668:
2662:
2659:
2657:
2654:
2652:
2649:
2647:
2644:
2642:
2639:
2637:
2634:
2633:
2631:
2629:
2625:
2619:
2616:
2614:
2611:
2609:
2606:
2604:
2601:
2600:
2598:
2596:
2592:
2589:
2587:
2583:
2580:
2578:
2574:
2569:
2562:
2557:
2555:
2550:
2548:
2543:
2542:
2539:
2523:
2520:
2518:
2515:
2513:
2510:
2508:
2505:
2504:
2502:
2498:
2486:
2483:
2481:
2478:
2477:
2476:
2475:
2471:
2467:
2464:
2462:
2459:
2457:
2454:
2453:
2452:
2451:
2447:
2446:
2444:
2442:
2438:
2432:
2431:Myelophthisic
2429:
2427:
2424:
2422:
2418:
2417:
2415:
2412:
2410:
2406:
2403:
2401:
2398:
2393:
2389:
2379:
2376:
2375:
2372:
2369:
2367:
2364:
2363:
2358:
2355:
2354:
2353:
2350:
2348:
2345:
2344:
2339:
2336:
2335:
2334:
2333:
2329:
2328:
2319:
2316:
2312:
2309:
2308:
2307:
2304:
2302:
2299:
2297:
2294:
2293:
2291:
2289:
2285:
2282:
2280:
2276:
2270:
2267:
2263:
2260:
2259:
2258:
2255:
2251:
2248:
2247:
2246:
2243:
2241:
2237:
2235:
2232:
2230:
2226:
2223:
2219:
2216:
2214:
2211:
2209:
2206:
2205:
2204:
2201:
2199:
2195:
2194:
2189:
2186:
2184:
2181:
2179:
2176:
2175:
2174:
2173:
2169:
2167:
2164:
2162:
2158:
2157:
2155:
2153:
2149:
2146:
2143:
2138:
2134:
2126:
2123:
2122:
2121:
2117:
2114:
2113:
2108:
2105:
2104:
2103:
2099:
2096:
2095:
2093:
2091:
2087:
2084:
2082:
2078:
2075:
2071:
2061:
2058:
2057:
2055:
2053:
2049:
2046:
2042:
2038:
2034:
2027:
2022:
2020:
2015:
2013:
2008:
2007:
2004:
1998:
1995:
1993:
1990:
1989:
1979:
1975:
1974:
1970:
1968:
1964:
1963:
1959:
1957:
1953:
1952:
1948:
1947:
1944:
1940:
1933:
1929:
1928:
1924:
1922:
1918:
1917:
1913:
1911:
1907:
1906:
1902:
1898:
1896:
1892:
1891:
1887:
1883:
1882:
1879:
1874:
1870:
1863:
1854:
1850:
1846:
1842:
1838:
1834:
1826:
1823:
1817:
1814:
1809:
1805:
1801:
1797:
1793:
1789:
1786:(2): 135–41.
1785:
1781:
1774:
1771:
1765:
1759:
1755:
1748:
1745:
1740:
1736:
1732:
1728:
1725:(2): 118–41.
1724:
1720:
1713:
1710:
1705:
1701:
1697:
1693:
1689:
1685:
1681:
1677:
1670:
1668:
1666:
1662:
1657:
1653:
1648:
1643:
1640:(4): 539–43.
1639:
1635:
1631:
1624:
1622:
1620:
1616:
1611:
1605:
1601:
1600:
1592:
1590:
1588:
1586:
1584:
1580:
1575:
1569:
1566:
1550:
1543:
1536:
1533:
1522:on 2012-12-23
1521:
1517:
1510:
1507:
1502:
1498:
1494:
1490:
1486:
1482:
1478:
1474:
1466:
1464:
1462:
1460:
1456:
1451:
1449:0-7216-0187-1
1445:
1441:
1434:
1431:
1424:
1420:
1417:
1415:
1412:
1410:
1409:Spherocytosis
1407:
1406:
1402:
1397:
1394:
1391:
1388:
1385:
1382:Experimental
1381:
1380:
1379:
1373:
1368:
1365:
1362:
1359:
1354:
1353:glycosylation
1350:
1347:
1346:
1344:
1343:Hyperglycemia
1341:
1337:
1334:
1331:
1327:
1326:megaloblastic
1323:
1320:
1319:
1315:
1311:Complications
1310:
1305:
1302:
1301:
1300:
1294:
1290:
1288:
1284:
1283:
1282:
1276:
1273:
1272:
1271:
1268:
1266:
1265:meningococcus
1262:
1261:
1256:
1248:
1246:
1241:
1238:
1235:
1232:
1230:
1226:
1225:
1224:
1221:
1214:
1212:
1210:
1206:
1202:
1195:
1193:
1190:
1189:iron overload
1186:
1182:
1178:
1170:
1169:Gold standard
1166:
1162:
1159:
1156:
1153:
1148:
1145:
1141:
1138:
1133:
1129:
1126:
1123:
1120:
1119:
1118:
1115:
1112:
1109:
1106:
1103:
1100:
1096:
1093:
1092:
1091:
1085:
1080:
1076:
1073:
1071:
1068:
1065:
1062:
1058:
1054:
1050:
1049:reticulocytes
1046:
1042:
1038:
1034:
1030:
1028:
1025:
1024:
1023:
1017:
1012:
1010:
1008:
1004:
1003:cholecystitis
1000:
996:
992:
988:
984:
976:
973:
970:
969:
968:
962:
960:
958:
954:
950:
946:
941:
937:
936:fenestrations
933:
924:
920:
917:
913:
910:
906:
902:
901:
900:
896:
894:
890:
881:
879:
873:
870:
869:microvesicles
866:
862:
861:
860:
858:
850:
844:Least Common
843:
841:
839:
835:
831:
823:
821:
817:
813:
806:
802:
799:
796:
792:
790:
787:
786:
782:
780:
778:
774:
766:
764:
760:
753:
749:
746:
743:
735:
731:
727:
723:
721:
718:
717:
713:
711:
709:
705:
697:
695:
691:
684:
680:
677:
674:
672:
669:
668:
665:
663:
660:
656:
648:
646:
642:
638:
631:
627:
624:
621:
618:
616:
613:
612:
605:
598:
595:
592:
589:
586:
583:
582:
576:
574:
570:
562:
560:
556:
549:
545:
542:
538:
535:
532:
528:
525:
524:
521:
517:
514:
511:
508:
505:
502:
501:
496:
493:
485:
483:
481:
477:
472:
471:
469:
458:
455:
453:
452:
448:
446:
443:
440:
439:
435:
432:
430:
429:
425:
423:
420:
417:
416:
412:
409:
407:
406:
402:
400:
397:
394:
393:
389:
386:
384:
383:
379:
377:
374:
371:
370:
366:
363:
361:
360:
356:
354:
351:
348:
347:
343:
340:
337:
334:
331:
330:
326:
319:
316:
314:
310:
306:
302:
298:
294:
286:
281:
279:
277:
272:
268:
260:
258:
255:
253:
250:
248:
245:
242:
239:
238:
237:
231:
229:
227:
223:
222:
213:
211:
209:
204:
200:
196:
192:
188:
184:
180:
176:
172:
168:
164:
160:
156:
152:
148:
145:secondary to
144:
140:
136:
131:
129:
125:
119:
117:
113:
109:
105:
104:spherocytosis
101:
97:
93:
89:
85:
77:
74:
72:
68:
63:
59:
54:
50:
46:
41:
33:
32:Spherocytosis
19:
3431:
3413:
3333:
3321:
3302:
3273:
3162:Microtubules
2568:Cytoskeletal
2480:normochromic
2472:
2448:
2330:
2244:
2238:
2196:
2170:
2159:
2052:Polycythemia
1971:
1960:
1949:
1925:
1914:
1899:
1884:
1836:
1832:
1825:
1816:
1783:
1779:
1773:
1768:8th edition.
1753:
1747:
1722:
1718:
1712:
1679:
1675:
1637:
1633:
1598:
1568:
1556:. Retrieved
1549:the original
1535:
1524:. Retrieved
1520:the original
1509:
1476:
1472:
1439:
1433:
1384:gene therapy
1377:
1298:
1285:
1280:
1269:
1258:
1252:
1242:
1236:
1227:
1222:
1218:
1199:
1174:
1160:
1154:
1116:
1110:
1104:
1094:
1089:
1021:
980:
966:
953:splenomegaly
928:
897:
885:
877:
867:-containing
854:
837:
833:
829:
827:
819:
815:
811:
809:
783:Less Common
776:
772:
770:
762:
758:
756:
707:
703:
701:
693:
689:
687:
661:
658:
654:
652:
644:
640:
636:
634:
579:Most Common
572:
568:
566:
558:
554:
552:
541:cytoskeleton
519:
489:
473:
465:
464:
459:Protein-4.2
449:
426:
403:
380:
357:
290:
264:
235:
219:
217:
214:Epidemiology
199:reticulocyte
178:
135:splenomegaly
132:
120:
87:
83:
82:
3394:plakophilin
3357:desmoplakin
3323:plakoglobin
3077:Laminopathy
2973:Monilethrix
2821:keratoderma
2694:Tropomyosin
2586:Myofilament
2485:hypochromic
2441:Blood tests
2203:Thalassemia
2161:enzymopathy
2090:Nutritional
1951:MedlinePlus
1231:(operative)
1229:Splenectomy
1205:splenectomy
1095:Coombs test
1079:Coombs test
1057:haptoglobin
1013:Diagnostics
945:phagocytose
940:macrophages
830:protein-4.2
812:protein-4.2
793:Failure of
789:Protein-4.2
734:bicarbonate
520:(In Order)
515:Likelihood
313:protein 4.2
257:Protein-4.2
191:splenectomy
183:blood smear
167:gallbladder
151:kernicterus
98:causes the
48:Other names
3448:Categories
3410:centrosome
3027:Peripherin
2466:macrocytic
2461:microcytic
2456:normocytic
2419:Acquired:
2405:Hereditary
2172:glycolysis
2152:Hereditary
1526:2012-07-03
1425:References
1419:Hematology
1363:Leg ulcer.
1304:Folic acid
1201:Ultrasound
995:gallstones
907:risk (see
732:(Cl) with
433:17q21-q22
387:14q22-q23
195:hemoglobin
149:and acute
128:expression
76:Hematology
3228:Tauopathy
2817:keratosis
2745:Fibrillin
2137:Hemolytic
1962:eMedicine
1255:influenza
1215:Treatment
840:(bottom).
834:inability
826:Caption:
822:(bottom).
816:inability
808:Caption:
769:Caption:
755:Caption:
740:) across
700:Caption:
686:Caption:
659:failed or
651:Caption:
633:Caption:
599:Hemolysis
596:Fragility
565:Caption:
551:Caption:
311:protein,
276:mutations
226:mutations
203:bilirubin
175:infection
163:bilirubin
155:bilirubin
96:phenotype
71:Specialty
3255:Spectrin
3247:Membrane
3088:Dunnigan
2671:Troponin
2395:(mostly
2392:Aplastic
2332:membrane
2279:Acquired
2240:membrane
2140:(mostly
2033:Diseases
1973:Orphanet
1967:med/2147
1853:21238662
1800:18727072
1739:15071790
1704:10926437
1696:18940465
1493:15287938
1403:See also
1374:Research
1039:(MCHC),
983:jaundice
730:chloride
726:exchange
527:Spectrin
492:spectrin
367:Ankyrin
293:spectrin
241:Spectrin
232:Etiology
92:mutation
3378:plectin
3295:Catenin
3275:Ankyrin
3171:Kinesin
3079:: LMNA
2762:Filamin
2570:defects
1932:D013103
1808:7339411
1656:9887280
1501:5870305
1196:Imaging
1047:(RBC),
1043:(RDW),
1035:(MCV),
1009:, etc.
911:below).
857:alveoli
714:Common
704:ankyrin
690:ankyrin
671:Ankyrin
569:alpha-1
555:alpha-1
531:Alpha-1
364:8p11.2
305:ankyrin
247:Ankyrin
181:. On a
143:hypoxia
139:fatigue
126:in its
108:rupture
3194:Dynein
3122:LEMD3
3000:Desmin
2628:Myosin
2613:DFNA20
2397:normo-
2142:normo-
2116:Macro-
2098:Micro-
2081:Anemia
1956:000530
1921:182900
1851:
1806:
1798:
1760:
1737:
1702:
1694:
1676:Lancet
1654:
1606:
1558:2 July
1499:
1491:
1446:
1414:Anemia
1143:liver.
1131:value.
1063:(LDH).
991:anemia
905:sepsis
865:band 3
720:Band-3
641:failed
456:15q15
445:612690
428:SLC4A1
422:612653
399:270970
376:182870
353:182900
341:Locus
309:band 3
147:anemia
137:, and
112:spleen
3350:Other
3221:Other
3112:LMNB
2969:KRT86
2965:KRT83
2961:KRT81
2951:KRT18
2941:KRT17
2931:KRT14
2921:KRT13
2911:KRT12
2901:KRT10
2851:KRT2E
2738:Other
2717:Titin
2595:Actin
2500:Other
2229:trait
2218:delta
2208:alpha
1910:282.0
1895:D58.0
1804:S2CID
1700:S2CID
1552:(PDF)
1545:(PDF)
1497:S2CID
451:EPB42
441:HS-5
418:HS-4
410:1q21
395:HS-3
372:HS-2
349:HS-1
338:Gene
297:alpha
3415:PCNT
3137:LBR
3016:GFAP
2891:KRT8
2881:KRT5
2871:KRT4
2861:KRT3
2844:IHCM
2829:KRT1
2288:AIHA
2213:beta
1927:MeSH
1916:OMIM
1905:9-CM
1849:PMID
1796:PMID
1758:ISBN
1735:PMID
1692:PMID
1652:PMID
1604:ISBN
1560:2012
1489:PMID
1444:ISBN
1263:and
1177:iron
916:heme
736:(HCO
655:Beta
637:Beta
468:OMIM
405:SPTA
382:SPTB
359:ANK1
333:OMIM
301:beta
299:and
201:and
3335:GAN
3304:APC
2827:):
2806:1/2
2035:of
1978:822
1901:ICD
1886:ICD
1841:doi
1788:doi
1727:doi
1684:doi
1680:372
1642:doi
1481:doi
1477:126
989:),
889:DVT
795:ATP
728:of
643:or
303:),
269:or
3450::
3412::
3396::
3380::
3359::
3277::
3257::
3049::
3029::
3018::
3002::
2823:,
2819:,
2798:IF
2407::
2118::
2100::
1976::
1965::
1954::
1930::
1919::
1908::
1893::
1890:10
1847:.
1837:46
1835:.
1802:.
1794:.
1784:76
1782:.
1733:.
1723:41
1721:.
1698:.
1690:.
1678:.
1664:^
1650:.
1638:24
1636:.
1632:.
1618:^
1582:^
1495:.
1487:.
1475:.
1458:^
1211:.
1187:,
1167:.
1059:,
1055:,
1051:,
1005:,
959:.
744:.
543:.
533:)
335:*
307:,
278:.
228:.
210:.
197:,
185:,
130:.
118:.
88:HS
3422:)
3418:(
3342:)
3338:(
3330:)
3326:(
3070:5
3040:4
2993:3
2975:)
2971:(
2967:/
2963:/
2957:)
2953:(
2947:)
2943:(
2937:)
2933:(
2927:)
2923:(
2917:)
2913:(
2907:)
2903:(
2897:)
2893:(
2887:)
2883:(
2877:)
2873:(
2867:)
2863:(
2857:)
2853:(
2815:(
2560:e
2553:t
2546:v
2399:)
2242::
2227:/
2200::
2163::
2144:)
2073:↓
2044:↑
2025:e
2018:t
2011:v
1903:-
1888:-
1878:D
1855:.
1843::
1810:.
1790::
1766:.
1741:.
1729::
1706:.
1686::
1658:.
1644::
1612:.
1576:.
1562:.
1529:.
1503:.
1483::
1452:.
1332:.
1295:.
1277:.
1247:)
1139:.
1101:)
1081:)
738:3
529:(
295:(
86:(
34:.
20:)
Text is available under the Creative Commons Attribution-ShareAlike License. Additional terms may apply.
