122:
108:
globin gene cluster, or the Îł gene promoter region. In addition HbF levels are influenced by polymorphisms in the BCL11A gene and in the MYB gene enhancer. In HPFH the percentage of HbF varies from 0.8-1.0% to about 30% of the total hemoglobin, but levels as high as 100% can be seen in homozygotes
83:
In persons with sickle cell disease, high levels of fetal hemoglobin as found in a newborn or as found abnormally in persons with hereditary persistence of fetal hemoglobin, the HbF causes the sickle cell disease to be less severe. In essence the HbF inhibits polymerization of HbS. A similar
432:
417:
230:
Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009-05-28). Robbins and Cotran
Pathologic Basis of Disease, Professional Edition: Expert Consult - Online (Robbins Pathology) (Kindle Locations 33411-33412). Elsevier Health. Kindle
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Stadhouders, R; Aktuna, S; Thongjuea, S; Aghajanirefah, A; Pourfarzad, F; van Ijcken, W; Lenhard, B; Rooks, H; Best, S; Menzel, S; Grosveld, F; Thein, SL; Soler, E (April 2014).
1014:
347:
Friedman S, Schwartz E (January 1976). "Hereditary persistence of foetal haemoglobin with beta-chain synthesis in cis position (Ggamma-beta+-HPFH) in a negro family".
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833:
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758:
828:
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164:, and is selected for in populations with a high prevalence of these conditions (which in turn are often selected for in areas where
809:
780:
712:
840:
773:
479:
819:
867:
724:
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640:
63:, HbF) production continues well into adulthood, disregarding the normal shutoff point after which only adult-type
741:
731:
719:
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707:
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650:
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356:
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942:
687:
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436:
156:
About 10% of the population has an HbF level >1.0%. HPFH may alleviate the severity of certain
75:
The condition is asymptomatic, and is only noticed when screening for other hemoglobin disorders.
974:
947:
552:
380:
979:
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923:
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499:
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275:
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441:
300:"HBS1L-MYB intergenic variants modulate fetal hemoglobin via long-range MYB enhancers"
121:
1003:
208:
191:
88:. Approximately 40% of the hemoglobin is in the HbS form while the rest is in normal
168:
is endemic). Thus, it has been found to affect people of
African and Greek descent.
514:
384:
89:
60:
665:
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161:
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464:
426:
64:
40:
192:"Discovering the genetics underlying foetal haemoglobin production in adults"
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376:
101:
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165:
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468:
116:
243:"Regulation of the fetal hemoglobin silencing factor BCL11A"
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form. The HbA form interferes with HbS polymerization.
399:
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84:mechanism occurs with persons who have sickle cell
34:
26:
21:
480:
8:
629:Glucose-6-phosphate dehydrogenase deficiency
30:Hereditary persistence of foetal haemoglobin
185:
183:
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55:) is a benign condition in which increased
985:Hereditary persistence of fetal hemoglobin
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610:
548:
539:
510:
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247:Annals of the New York Academy of Sciences
49:Hereditary persistence of fetal hemoglobin
22:Hereditary persistence of fetal hemoglobin
18:
1015:Disorders of globin and globulin proteins
937:Mean corpuscular hemoglobin concentration
759:Warm antibody autoimmune hemolytic anemia
323:
274:
207:
177:
304:The Journal of Clinical Investigation
241:Basak, A; Sankaran, VG (March 2016).
7:
646:triosephosphate isomerase deficiency
801:paroxysmal nocturnal hemoglobinuria
769:Donath–Landsteiner hemolytic anemia
14:
810:Microangiopathic hemolytic anemia
781:Mixed autoimmune hemolytic anemia
190:Thein, SL; Menzel, S (May 2009).
841:Hemolytic disease of the newborn
209:10.1111/j.1365-2141.2009.07650.x
120:
774:Paroxysmal cold hemoglobinuria
196:British Journal of Haematology
1:
1010:Hereditary hemolytic anemias
725:Southeast Asian ovalocytosis
713:Minkowski–Chauffard syndrome
109:for delta beta thalassemia.
1031:
834:Drug-induced nonautoimmune
815:Thrombotic microangiopathy
641:pyruvate kinase deficiency
820:Hemolytic–uremic syndrome
732:Hereditary stomatocytosis
720:Hereditary elliptocytosis
708:Hereditary spherocytosis
913:Mean corpuscular volume
877:Diamond–Blackfan anemia
829:Drug-induced autoimmune
764:Cold agglutinin disease
570:Plummer–Vinson syndrome
565:Iron-deficiency anemia
129:This section is empty.
100:HPFH can be caused by
884:Pure red cell aplasia
651:hexokinase deficiency
889:Sideroblastic anemia
697:Hemoglobin C disease
583:Megaloblastic anemia
67:should be produced.
688:Sickle cell disease
361:1976Natur.259..138F
259:2016NYASA1368...25B
79:Sickle cell disease
975:Sulfhemoglobinemia
267:10.1111/nyas.13024
158:hemoglobinopathies
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996:
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980:Reticulocytopenia
970:Methemoglobinemia
958:
957:
849:
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789:
788:
588:Pernicious anemia
531:
530:
523:Polycythemia vera
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16:Medical condition
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661:hemoglobinopathy
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369:10.1038/259138a0
355:(5539): 138–40.
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131:You can help by
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57:fetal hemoglobin
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500:red blood cells
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310:(4): 1699–710.
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872:Fanconi anemia
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404:Classification
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396:External links
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140:September 2021
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894:Myelophthisic
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202:(4): 455–67.
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984:
943:normochromic
935:
911:
793:
701:
659:
633:
622:
515:Polycythemia
446:
431:
416:
352:
348:
342:
307:
303:
293:
253:(1): 25–30.
250:
246:
236:
226:
199:
195:
162:thalassemias
155:
152:Epidemiology
137:
133:adding to it
128:
105:
99:
85:
82:
74:
71:Presentation
61:hemoglobin F
52:
48:
47:
948:hypochromic
904:Blood tests
666:Thalassemia
624:enzymopathy
553:Nutritional
27:Other names
1004:Categories
929:macrocytic
924:microcytic
919:normocytic
882:Acquired:
868:Hereditary
635:glycolysis
615:Hereditary
172:References
65:hemoglobin
41:Pediatrics
600:Hemolytic
113:Diagnosis
102:mutations
36:Specialty
858:(mostly
855:Aplastic
795:membrane
742:Acquired
703:membrane
603:(mostly
496:Diseases
334:24614105
285:26963603
231:Edition.
218:19344402
385:4183236
377:1246351
357:Bibcode
325:3973089
276:4870126
255:Bibcode
166:malaria
104:in the
860:normo-
605:normo-
579:Macro-
561:Micro-
544:Anemia
453:141749
383:
375:
349:Nature
332:
322:
283:
273:
216:
96:Causes
963:Other
692:trait
681:delta
671:alpha
442:282.7
427:D56.4
381:S2CID
86:trait
751:AIHA
676:beta
448:OMIM
437:9-CM
373:PMID
330:PMID
281:PMID
251:1368
214:PMID
160:and
53:HPFH
498:of
433:ICD
418:ICD
365:doi
353:259
320:PMC
312:doi
308:124
271:PMC
263:doi
204:doi
200:145
135:.
90:HbA
1006::
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180:^
862:)
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663::
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536:↓
507:↑
488:e
481:t
474:v
435:-
420:-
410:D
387:.
367::
359::
336:.
314::
287:.
265::
257::
220:.
206::
142:)
138:(
106:β
59:(
51:(
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