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Published on: June 10, 2017
Haemophagocytic lymphohistiocytosis (HLH) with concurrent Hodgkin's Disease
Vanessa Ambrose Fistus1, Mohiuddin Sharief2, Anita Sarma3
1Acute Medicine, Royal Preston Hospital, Preston, UK vanessaambrose93@gmail.com.
Haemophagocytic lymphohistiocytosis (HLH) is a condition of uncontrolled immune activation caused by genetic defects or triggered by infections, malignancies, autoimmune diseases, drugs, pregnancy or post-transplant immunosuppression. This case describes a young man presenting with clinical jaundice and abnormal blood tests, including raised inflammatory markers, abnormal liver function, low haemoglobin (65 g/L) and a low white blood cell count (3.08×109/L). He met five out of eight HLH criteria and tested positive for Epstein-Barr virus (EBV) PCR (58 011 IU/mL). His bone marrow biopsy showed EBV-driven Hodgkin's lymphoma. He was initially treated with the HLH-94 protocol and later switched to ABVD chemotherapy (adriamycin, bleomycin, vinblastine and dacarbazine). He steadily recovered despite a prolonged hospital stay. He was discharged to complete his remaining cycles of chemotherapy as an outpatient with a plan of having a positron emission tomography (PET) scan after two cycles of chemotherapy.
Haemophagocytic lymphohistiocytosis (HLH) is a condition of uncontrolled immune activation caused by genetic defects or triggered by infections, malignancies, autoimmune diseases, drugs, pregnancy or post-transplant immunosuppression. This case describes a young man presenting with clinical jaundice and abnormal blood tests, including raised inflammatory markers, abnormal liver function, low haemoglobin (65 g/L) and a low white blood cell count (3.08×109/L). He met five out of eight HLH criteria and tested positive for Epstein-Barr virus (EBV) PCR (58 011 IU/mL). His bone marrow biopsy showed EBV-driven Hodgkin's lymphoma. He was initially treated with the HLH-94 protocol and later switched to ABVD chemotherapy (adriamycin, bleomycin, vinblastine and dacarbazine). He steadily recovered despite a prolonged hospital stay. He was discharged to complete his remaining cycles of chemotherapy as an outpatient with a plan of having a positron emission tomography (PET) scan after two cycles of chemotherapy.
