Hemophagocytic lymphohstiocytosis: A Case Report

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Hemophagocytic lymphohstiocytosis: A Case Report

Journal Title: Biomedical Journal of Scientific & Technical Research (BJSTR) - Year 2017, Vol 1, Issue 2

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a life threatening hyper inflammatory syndrome occurring due to ineffective immune process. It can be genetic (familial form) or secondary (acquired form). We present a case of HLH in a 50 year old male who presented with pancytopenia and huge platelet clumps on peripheral smear which subsequently leads to diagnosis after correlation with clinical, hematological and biochemical findings. Hemophagocytic syndrome with special mention to its secondary form is a rare syndrome which occurs after strong immunologic activation and release of cytokines characterized by unrestricted activation of T lymphocytes and macrophages invariably leading to death in absence of treatment [1,2]. It is associated with certain clinical and hematological abnormalities of which pancytopenia, hyper ferritinaemia, hepatosplenomegaly, fever and hemophagocytosis in bone marrow were observed in our case. Case Report A Fifty year old male patient presented with decreased appetite since 10 to 15 days and dyspnoea on exertion associated with cough. The patient was non alcoholic and non smoker. On examination, the patient was febrile, oriented and a wheeze was heard on right lower zone. Chest X-ray showed a diffuse haziness on right lower zone. His ultrasonography of abdomen revealed hepatosplenomegaly with dilated portal vein, splenic artery and ascitis. The liver function tests were altered and revealed a high bilirubin= 3.4 mg/dl (N= 0.20 -1.0 mg/dl), direct bilirubin = 2.90 mg/dl (N= 0- 0.2 mg/dl), SGOT= 136 U/L (5-40 U/L), SGPT= 76 U/L (5-35 U/L) and Alkaline phosphatase = 621 U/L (28-112 U/L). Renal function tests and other biochemical investigations were unremarkable. Dengue serology and malaria antigen detection tests were negative. A clinical diagnosis of? Hepatitis? Lower respiratory tract infection?? Acute respiratory distress syndrome was made. The patient was non reactive for HbS antigen and HCV antibody. The hematological investigations showed pancytopenia (Hb = 6.0 g/dl, TLC= 2500/mm3 and platelets= 50,000 cu.mm). On peripheral smear examination, RBCs were predominantly microcytic hypo chromic revealing mild degree of anisopoikilocytosis. The differential leucocyte count was polymorphs - 69%, lymphocytes- 25%, monocytes-6. An interesting finding was presence of large number of platelet clumps or aggregates (Figure 1). The sample was repeated to rule out EDTA effect on platelets, but this feature persisted and also showed few platelet clumps engulfing WBCs. On account of this, a prothombin time and activated partial thromboplastin time was ordered. Both came out to be high. PT = 22 sec (N= 11-16 secs) and APTT = 36 sec (N =30-32 secs). The peripheral smear was examined again and it was observed that many of the polymorphs and monocytes revealed cytoplasmic vacuolation suggestive of some infectious etiology and subsequently blood culture report was positive for pseudomonas aeroginosa sensitive to meropenem, imipenem, amikacin, ceftriaxone, tobramycin and piperacillin. The sputum was also sent for culture and came out to be positive for candida. Based on all these investigations the patient was diagnosed as suffering from fungal pneumonia with hepatitis associated with portal hypertension along with sepsis and was treated for the same but on subsequent investigations, pancytopenia remained unresolved. Hence, a bone marrow examination was done. The aspirate was particulate and norm cellular for age. The myeloid and elytroid precursors were well preserved. Megakaryocytes were adequate in number. In addition to this, there was a prominence of macrophages showing phagocytosis of hematopoietic cells particularly platelets and erythroid precursors (Figure 2). The plasma cells were also present in significant numbers. A serum ferritin level was ordered which came out to be significantly high= 1650mg/dl (N=24-336mg/dl).The serum triglyceride levels were marginally high (152mg/dl) (N=40-149 mg/dl).Hence it was found that the patient fulfills five criteria of Hemophagocytic syndrome which are fever, splenomegaly & hepatomegaly with hepatitis like features, pancytopenia, hyperferritenemia and evidence of hemophagocytosis on bone marrow. The increased PT, APTT values, presence of blood clots and positive blood culture report for pseudomonas were supporting evidence of sepsis and the patient likely being in DIC. In our case this was the presumptive cause of hemophagocytic syndrome. This case is different because it was the repeated appearance of platelet aggregates on peripheral smear with pancytopenia and hepatosplenomegaly that raised the doubt of a Hemophagocytic syndrome which was further confirmed by bone marrow examination and other biochemical investigations.

Authors and Affiliations

Kriti Chauhan, Ridhima Auplish, Kshitiz Vashista

Keywords

HLH Pancytopenia Platelet Clumps Biomedical Research Biomedres Open access publishers Peer reviewed journals Special issues Biomedres â€“ ebook publishers Biomedres â€“ video publishers Biomedical Journal of Scientific & Technical Research International academic journals Open access medical and clinical research publication The Complex world of Bio-Medical Sciences Health Sciences and Bio informatics Physical Activity and Computational Biology Life Sciences in relation with Technical Advancement Special issue publishers Effective Publishers Trendy online Publishers Popular online Publishers Biomedical Research papers publishers Biomedical sciences and Technical papers repository Online Publishers

EP ID EP569271
DOI 10.26717/BJSTR.2017.01.000232
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