Macrophage Activation Syndrome Associated With Hodgkin Lymphoma and Hiv Infection: An Uncommon Association
Title: Macrophage Activation Syndrome associated with Hodgkin lymphoma and HIV infection: an uncommon association.
Aim: To present a rare association of Macrophage Activation Syndrome with Hodgkin lymphoma in a HIV- infected patient.
Introduction: Macrophage Activation Syndrome (MAS) is characterized by proliferation of benign macrophages, leading to extensive phagocytosis of haematopoietic cells and alteration in the cytotoxic function of T-cells & natural killer cells and it belongs to hemophagocytic lymphohistiocytosis (HLH) disorders. Here we report a patient already infected with HIV, who presented with initial manifestation of MAS like fever, hepatosplenomegaly, pancytopenia with raised triglycerides and ferritin. Later on, through biopsy, the patient was diagnosed to be a case of Hodgkin lymphoma also.
Methods: A 35 year old female, known case of HIV-infection/AIDS (on TLE), presented with fever of duration 15 days. On examination, patient was found to have pancytopenia (Hb 5.4 g/dL, TLC 1300/cumm, platelets 40000/cumm). Reticulocyte count was 0.5%. There was hypertriglyceridemia (TG ˃ 500 mg/dL) and raised LDH .Also, there was mild derangement of SGOT/PT and raised SAP and gamma-glutamyltransferase. Serum ferritin was also raised (˃ 2000 ng/ml). Other biochemical investigations were within normal limits .On the basis patient being a known case of HIV infection/AIDS, presenting with fever and hepatosplenomegaly, raised triglycerides and raised serum ferritin, a diagnosis of Macrophage Activation Syndrome was made (5 out of 8 diagnostic criteria fulfilled). Bone marrow biopsy was suggestive of chronic myeloid neoplasm- idiopathic myelofibrosis (grade 2). The patient also consulted another tertiary care institute of the region with better facilities, where bone marrow biopsy (presence of classic Reed-Sternberg cells) and immunohistochemistry (positivity for CD30 and PAX5 in Reed-Sternberg cells) was suggestive of infiltration by Hodgkin lymphoma. Patient was treated for lymphoma by AVD (Adriamycin, Vinblastine and Dacarbazine) regimen. PET scan was done and showed the evidence of multiple FDG avid, enlarged lymph node regions, such as bilateral cervical, axillary & left iliac region. With the treatment, patient responded well and a repeat PET scan after few months of the treatment, showed no evidence of metabolically active residual/recurrent disease. But her pancytopenia still persisted till the last follow up. Thus a final diagnosis of Hodgkin lymphoma associated with MAS in the setting of HIV infection was made.
Discussion: The Macrophage Activation Syndrome is generally associated with infections such as HIV, EBV & tuberculosis, autoimmune diseases and malignancies. In our patient, possibility of Hodgkin lymphoma presenting initially as MAS is high because her viral load of HIV were very low. Autoimmune diseases, tuberculosis were ruled out by relevant clinical features and investigstions. The patient responded well to the treatment for the Hodgkin Lymphoma, but manifestation of pancytopenia were still there, may be due to effects of chemotherapy or ongoing MAS in chronic form.
Conclusion: Malignant diseases such as Hodgkin Lymphoma, can present with the manifestation of macrophage activation syndrome initially. A diagnosis of Macrophage Activation Syndrome should be kept in mind, in the setting of chronic pancytopenia.