Keywords
HbSDPunjab
hemoglobinopathy
high performance liquid chromatography
How to Cite
Abstract
Hemoglobinopathies constitute one of the most common inherited hematological disorders in the world with an increasing global disease burden each year. One among them is sickle cell disease with diverse genotypes and wide phenotypic heterogenity. Many subgroups exist within the umbrella of sickle cell disease. Hb S/DPunjab, a rare hemoglobinopathy, is one of them, mimics sickle cell disease, and is discussed in the present study. We describe one such unusual clinical case of a young child who presented with intermittent fever and joint problems. The study case was found to have Hb S/DPunjab by high performance liquid chromatography. Clinical and hematological details of this rare condition is only briefly discussed in the literature. Precise diagnosis can be made using high performance liquid chromatography in conjunction with family studies.
References
Rodak., Carr. Clinical hematology atlas. St. Louis: Elsevier; 2017.
Hassan M, Ali Al-Barazanchi Z, Abdulateef S, Jaber R. Double heterozygosity for hemoglobin S and D Punjab in Basra, Iraq: A Clinical and hematological study of 42 patients. Journal of Applied Hematology. 2019;10(4):134.
Nogueira R, Leite C, Souza L, Barbosa A. Clinical and laboratory repercussions in patient with hemoglobin SD-Punjab disease: a case report. Jornal Brasileiro de Patologia e Medicina Laboratorial. 2017;53(5).
Randolph TR. Hemoglobinopathies (Structural Defects in Hemoglobin). In Rodak BF, Fritsma GA, and Doig K (eds.):Hematology: Clinical Principles and Applications, 3rd ed, St. Louis: Saunders, Elsevier, 2007:333-54.
Weatherall, D. J. The challenges of haemoglobinopathies in resource-poor countries. Br J Haematol. 2011; 154:736–744.
Brousse V, Rees D. Sickle cell disease: More than a century of progress. Where do we stand now?. Indian Journal of Medical Research. 2021;154(1):4.
Hockham C, Bhatt S, Colah R, Mukherjee M, Penman B, Gupta S et al. The spatial epidemiology of sickle-cell anaemia in India. Scientific Reports. 2018;8(1).
Cawein M. Hemoglobin S-D Disease. Annals of Internal Medicine. 1966;64(1):62.
Ali W, Jain M, Agarwal S, Kumar A. A Case of Hemoglobin Sickle-D Punjab. Indian Journal of Hematology and Blood Transfusion. 2019;36(1):205-207.
Rohilla Rakhi, Rohilla M, Malhotra P, Das R, Jain V et al. Compound Heterozygous Hemoglobin SD Disease Presenting as Sickle Cell Crisis in Pregnancy. Journal Of Woman's Reproductive Health. 2016; 1(2):25-28.
All material published in the journal HEMATOLOGÍA (electronic and print version) is transferred to the Argentinean Society of Hematology. In accordance with the copyright Act (Act 11 723), a copyright transfer form will be sent to the authors of approved works, which has to be signed by all the authors before its publication. Authors should keep a copy of the original since the journal is not responsible for damages or losses of the material that was submitted. Authors should send an electronic version to the email: revista@sah.org.ar