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
Related Articles
- EP ID EP569271
- DOI 10.26717/BJSTR.2017.01.000232
- Views 184
- Downloads 0
International Education Collaboration Using Social Media in Teaching and Learning Pain Management
All health-related professionals encounter patients with pain throughout their career. Pain is also a culturally moderated phenomenon. This project describes a journey of using social media to enhance the learning experi...
Isolation, Phylogenetic Analysis of the Microbial Community Associated with the Red Sea Sponge Ircinia Echinata and Biological Evaluation of their Secondary Metabolites
The present study was undertaken to provide information about bioactive compounds of the microbial community associated with Ircinia echinata marine sponge collected from the Red Sea. This work investigated the antioxida...
Biomarkers for Early Detection of Hypertensive Disorders in Pregnancy: Current Applications and Future Directions- The Role of Extracellular Matrix
Hypertensive disorders in pregnancy (HDP) are the most common medical complications, affecting 5–10% of pregnancies worldwide. Their early detection is critical for risk stratification and prevention of further pregnancy...
Unusual Locked Trigger Finger Due to Tophaceous Infiltration of Wrist Flexor Tendon
Tophi can present as a first sign of hyperuricemia but reports on the involvement of the flexor tendons of the hand is rare. Although tuberculous tenosynovitis is a more common etiology for swelling over the wrist, gout...
Chinese Urban Consumer’s Perception of Food-Safety Indicators
Households in 10 Chinese cities were asked to rank six food-safety indicators (venue, appearance, brand, certification, expiration date, and price) in importance for five food groups including meat, milk, vegetables, fru...