Title: Rare Case of Primary Hemophagocytic Lymphohistiocytosis: A Case Report

Authors: Dr Anurita Saigal, Dr Anchana Gulati, Dr Sudarshan Kumar Sharma, Dr Rajni Kaushik

 DOI: https://dx.doi.org/10.18535/jmscr/v7i4.45

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a fatal disease in which uncontrolled activation of immune system occurs. It is characterized by fever, pancytopenia, splenomegaly and hemophagocytosis in bone marrow, liver or lymph nodes. It has been associated with viral, bacterial, fungal and parasitic infection. An early diagnosis and treatment is the key to decrease the mortality .We report a case of hemophagocytic lymphohistiocytosis in a 39 year old male patient presenting with prolonged fever, pancytopenia, hepatosplenomegaly.

Keywords: Hemophagocytic lymphohistiocytosis; Macrophage, immune activation.

References

  1. Janka GE. Familial and acquired hemophagocytic lymphohistiocytosis. Annu Rev Med. 2012
  2. Otrock ZK, Eby CS: Clinical characteristics, prognostic factors, and outcomes of adult patients with hemophagocytic lymphohistiocytosis. Am J Hematol. 2014, 90:220-224. 1002/ajh.23911
  3. Nikiforow S, Berliner N: The unique aspects of presentation and diagnosis of hemophagocytic lymphohistiocytosis in adults. Hematology Am Soc Hematol Educ Program. 2015, 2015:183-189. 1182/asheducation-2015.1.183
  4. Risma K, Jordan MB. Hemophagocytic lymphohistiocytosis updates and evolving concepts. Curr Opin Pediatr. 2012;24(1):9‒15.
  5. Henter JI, Horne A, Arcio M, et al, HLH 2004,Diagnostic and therapeutic guidelines for Hemophagocytic Lymphohistiocytosis. Pediatric Blood Cancer 2007;48:124-131.

Corresponding Author

Dr Anurita Saigal

Department of Pathology, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India

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