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Hand-foot syndrome in sickle cell anemia

MedGen UID:
163110
•Concept ID:
C0852711
•
Disease or Syndrome
Synonyms: Sickle cell dactylitis; Sickle Cell Dactylitis
SNOMED CT: Hand-foot syndrome in sickle cell anemia (371104006); Sickle cell dactylitis (371104006)

Definition

A severe acute inflammatory response affecting the hands and feet of individuals with with sickle cell disease, sickle cell-hemoglobin C disease or sickle cell-beta-thalassemia. It is caused by vaso-occlusive episodes leading to ischemia and finally infarction of the distal portions of the extremities. Clinical signs of pain, swelling and tenderness of digits usually begin in early childhood and may be the initial manifestations of sickle cell anemia. Clinical course is self-limited with instances typically lasting a few weeks and occurring during sickling crises. An initial episode before the age of 1 strongly correlates with a more severe disease course. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVHand-foot syndrome in sickle cell anemia

Professional guidelines

PubMed

The presentation, management and prevention of crisis in sickle cell disease in Africa.
Fleming AF
Blood Rev 1989 Mar;3(1):18-28. doi: 10.1016/0268-960x(89)90022-2. PMID: 2650773

Recent clinical studies

Etiology

High rate of sickle cell anaemia in Sub-Saharan Africa underlines the need to screen all children with severe anaemia for the disease.
Kadima BT, Gini Ehungu JL, Ngiyulu RM, Ekulu PM, Aloni MN
Acta Paediatr 2015 Dec;104(12):1269-73. Epub 2015 Jun 4 doi: 10.1111/apa.13040. PMID: 25982693
Clinical features of sickle cell anaemia in northern nigerian children.
Ambe JP, Mava Y, Chama R, Farouq G, Machoko Y
West Afr J Med 2012 Apr-Jun;31(2):81-5. PMID: 23208475
The features of sickle cell disease in Saudi children.
el-Hazmi MA, Bahakim HM, al-Swailem AM, Warsy AS
J Trop Pediatr 1990 Aug;36(4):148-55. doi: 10.1093/tropej/36.4.148. PMID: 2213976
On the nature of sickle cell disease in the south-western province of Saudi Arabia.
el-Hazmi MA, Warsy AS
Acta Haematol 1986;76(4):212-6. doi: 10.1159/000206058. PMID: 2437751
Sickle cell anemia- clinical manifestations in 100 patients and review of the literature.
Karayalcin G, Rosner F, Kim KY, Chandra P, Aballi AJ
Am J Med Sci 1975 Jan-Feb;269(1):51-68. doi: 10.1097/00000441-197501000-00007. PMID: 1130436

Diagnosis

Clinical features of sickle cell anaemia in northern nigerian children.
Ambe JP, Mava Y, Chama R, Farouq G, Machoko Y
West Afr J Med 2012 Apr-Jun;31(2):81-5. PMID: 23208475
A clinico-epidemiological study of sickle cell anaemia in Saudi Arabia.
Mulik R, Butikofer A, Aramouni G, Munshi N, Iliff P
J Trop Pediatr 1991 Jun;37(3):100-5. doi: 10.1093/tropej/37.3.100. PMID: 1861279
The presentation, management and prevention of crisis in sickle cell disease in Africa.
Fleming AF
Blood Rev 1989 Mar;3(1):18-28. doi: 10.1016/0268-960x(89)90022-2. PMID: 2650773
Sickle cell disease in Sudan.
Bayoumi RA, Abu Zeid YA, Abdul Sadig A, Awad Elkarim O
Trans R Soc Trop Med Hyg 1988;82(1):164-8. doi: 10.1016/0035-9203(88)90298-2. PMID: 2459819
Sickle cell anemia- clinical manifestations in 100 patients and review of the literature.
Karayalcin G, Rosner F, Kim KY, Chandra P, Aballi AJ
Am J Med Sci 1975 Jan-Feb;269(1):51-68. doi: 10.1097/00000441-197501000-00007. PMID: 1130436

Therapy

The clinical characteristics of Congolese children and adolescents suffering from sickle-cell anemia are marked by the high frequencies of epistaxis compared to Western series.
Kazadi AL, Ngiyulu RM, Gini-Ehungu JL, Mbuyi-Muamba JM, Aloni MN
Pediatr Hematol Oncol 2019 Aug;36(5):267-276. Epub 2019 Jul 24 doi: 10.1080/08880018.2017.1365397. PMID: 31339399
Acquired Acrodermatitis Enteropathica Syndrome in a Kidney Transplant Receipt: A Case Report.
Bayraktar A, Bakkaloglu H, Demir E, Turkmen A, Azamat IF, Caliskan Y, Sari ŞO, Buyukbabani N, Baykal C, Aydin AE
Transplant Proc 2017 Apr;49(3):609-612. doi: 10.1016/j.transproceed.2017.02.030. PMID: 28340843
Sickle cell disease clinical phenotypes in children from South-Western, Nigeria.
Adegoke SA, Adeodu OO, Adekile AD
Niger J Clin Pract 2015 Jan-Feb;18(1):95-101. doi: 10.4103/1119-3077.146987. PMID: 25511352
Salmonella osteomyelitis and the hand-foot syndrome in sickle cell disease.
Bennett OM
J Pediatr Orthop 1992 Jul-Aug;12(4):534-8. doi: 10.1097/01241398-199207000-00023. PMID: 1613102
A clinico-epidemiological study of sickle cell anaemia in Saudi Arabia.
Mulik R, Butikofer A, Aramouni G, Munshi N, Iliff P
J Trop Pediatr 1991 Jun;37(3):100-5. doi: 10.1093/tropej/37.3.100. PMID: 1861279

Prognosis

The clinical characteristics of Congolese children and adolescents suffering from sickle-cell anemia are marked by the high frequencies of epistaxis compared to Western series.
Kazadi AL, Ngiyulu RM, Gini-Ehungu JL, Mbuyi-Muamba JM, Aloni MN
Pediatr Hematol Oncol 2019 Aug;36(5):267-276. Epub 2019 Jul 24 doi: 10.1080/08880018.2017.1365397. PMID: 31339399
Clinical features of sickle cell anaemia in northern nigerian children.
Ambe JP, Mava Y, Chama R, Farouq G, Machoko Y
West Afr J Med 2012 Apr-Jun;31(2):81-5. PMID: 23208475
Clinical profile of sickle cell disease in Yemeni children.
Al-Saqladi AW, Delpisheh A, Bin-Gadeem H, Brabin BJ
Ann Trop Paediatr 2007 Dec;27(4):253-9. doi: 10.1179/146532807X245634. PMID: 18053341
The presentation, management and prevention of crisis in sickle cell disease in Africa.
Fleming AF
Blood Rev 1989 Mar;3(1):18-28. doi: 10.1016/0268-960x(89)90022-2. PMID: 2650773
On the nature of sickle cell disease in the south-western province of Saudi Arabia.
el-Hazmi MA, Warsy AS
Acta Haematol 1986;76(4):212-6. doi: 10.1159/000206058. PMID: 2437751

Clinical prediction guides

Malaria, clinical features and acute crisis in children suffering from sickle cell disease in resource-limited settings: a retrospective description of 90 cases.
Aloni MN, Tshimanga BK, Ekulu PM, Ehungu JL, Ngiyulu RM
Pathog Glob Health 2013 Jun;107(4):198-201. doi: 10.1179/2047773213Y.0000000089. PMID: 23816320Free PMC Article
Early onset dactylitis associated with the occurrence of severe events in children with sickle cell anaemia. The Paediatric Cohort of Guadeloupe (1984-99).
Foucan L, Ekouevi D, Etienne-Julan M, Salmi LR, Diara JP; Paediatric Cohort of Guadeloupe
Paediatr Perinat Epidemiol 2006 Jan;20(1):59-66. doi: 10.1111/j.1365-3016.2006.00692.x. PMID: 16420342
The hand-foot syndrome in sickle-cell haemoglobinopathy.
Babhulkar SS, Pande K, Babhulkar S
J Bone Joint Surg Br 1995 Mar;77(2):310-2. PMID: 7706355
A clinico-epidemiological study of sickle cell anaemia in Saudi Arabia.
Mulik R, Butikofer A, Aramouni G, Munshi N, Iliff P
J Trop Pediatr 1991 Jun;37(3):100-5. doi: 10.1093/tropej/37.3.100. PMID: 1861279
Sickle cell disease in Sudan.
Bayoumi RA, Abu Zeid YA, Abdul Sadig A, Awad Elkarim O
Trans R Soc Trop Med Hyg 1988;82(1):164-8. doi: 10.1016/0035-9203(88)90298-2. PMID: 2459819

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