Haemophagocytic syndrome and elevated EBV load as initial manifestation of Hodgkin lymphoma in a HIV patient: case report and review of the literature

نویسندگان

  • Delphine Sculier
  • Thanh Doco-Lecompte
  • Mathieu Rougemont
  • Alexandra Calmy
چکیده

INTRODUCTION In HIV patients, haemophagocytic syndrome (HPS) may occur in the presence of cancer, concomitant viral infection, HIV primo-infection or at the initiation of highly active antiretroviral therapy (HAART). Hodgkin lymphoma remains a rare cause of HPS. We describe a case of HPS with very high Epstein Barr virus (EBV) load in a HIV patient as initial manifestation of Hodgkin lymphoma. MATERIALS AND METHODS A 29-year-old HIV positive man, successfully treated with HAART with an undetectable viral load and CD4 cells count of 438/µl, was admitted for high fever of unknown origin. Laboratory results showed a pancytopenia with haemoglobin at 82 g/l, lymphocyte count at 0.36G/l and platelets count at 47G/l; a highly elevated ferritine >7500 µg/l; increased lactate dehydrogenase at 885U/l and soluble IL2 receptor (CD25) >60 ng/ml. EBV load was measured and confirmed at 2,600,000 copies/ml. A PET-CT imaging showed diffuse elevated metabolic activity in the bone marrow and in two lesions in the spleen without lymphadenopathy. Bone marrow and liver biopsies revealed images of haemophagocytosis and lymphocyte depleted Hodgkin lymphoma. Treatment consisted in etoposid, steroids, and R-ABVD (rituximab, doxorubicin, bleomycin, vinblastine, dacarbazine) chemotherapy. The patient completed six cycles of chemotherapy. We reviewed the literature in PubMed with the following keywords: HPS, HIV, EBV, Hodgkin lymphoma. RESULTS We identified four publications and two reviews reporting cases of HPS associated with Hodgkin lymphoma in HIV patients with either a positive EBV load either the presence of encoded EBV RNA in tumour cells. Twenty-two cases (including one pediatric case) were described. Among adults, mostly men, the median age was <50 years and immune suppression was marked with a median CD4 cell count<100 cells/µl, even in patients receiving HAART. When measured, EBV load in the serum was high. Prognosis was poor with a high mortality despite adequate treatment consisting in steroids and chemotherapy, with or without etoposide (Table 1). CONCLUSIONS Our case report and the review of literature suggest that physicians should be aware of the association between EBV infection/reactivation and Hodgkin lymphoma as a cause of HPS in HIV patients, even if successfully treated with HAART. The pathogenesis of these three interrelated conditions (viral infection, oncogenesis and immunologic reaction) remains unclear.

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Acute renal failure due to renal lymphomatous infiltration as the initial manifestation

  A male patient with acute renal failure (ARF) due to large B-cell non-Hodgkin lymphoma infiltration of kidney is presented. The diagnosis was suspected because of coincidence of ARF and tumor lysis syndrome non-responsive to conservative renal therapies. A renal biopsy confirmed diagnosis and appropriate chemotherapy led to complete improvement of renal function.  

متن کامل

Primary Hepatic Burkitt’s Lymphoma in a Patient With Acquired Immunodeficiency Syndrome

  Primary non-Hodgkin lymphoma of liver is a very rare malignancy. Here we report a case of primary hepatic Burkitt’s lymphoma in a 34-year old man who was known case of acquired immunodeficiency syndrome (AIDS) and presented with weight loss, abdominal pain, and fever. Positive laboratory findings were mild anemia and elevated levels of lactate dehydrogenase (LDH) and alkaline phosphatase (AL...

متن کامل

Central Mucoepidermoid Carcinoma with Non-Hodgkin Lymphoma: A Case Report

Introduction: Central mucoepidermoid carcinoma (CMEC) is a rare primary intraosseous bony lesion. The mandible is more commonly affected than the maxilla. CMEC is frequently misdiagnosed radiographically and clinically as a benign odontogenic tumor or cyst. Case Presentation: We present a rare case with a secondary malignant neoplasm. A male patient was initially diagnosed with B-cell non-Hodg...

متن کامل

PRIMARY PANCREATIC LYMPHOMA: A CASE REPORT AND LITERATURE REVIEW

Primary pancreatic lymphoma is a rare extranodal manifestation of any histopathologic subtype of B cell non-Hodgkin's lymphoma that predominantly involves the pancreas. It comprises less than 0.2% of pancreatic malignancies and less than 0.7% of non-Hodgkin's lymphomas. This lymphoma is almost never suspected clinically. It is usually diagnosed by surgical exploration for suspected adenoca...

متن کامل

Malignant Lymphoma of Lacrimal Apparatus in the 7 Year Old Girl

Abstract Background Involvement of lacrimal apparatus by Non Hodgkin lymphoma as a first manifestation is extremely rare and only three cases have been reported in children. The purpose of this report is to determine the clinical characteristic of patient with primary involvement of lacrimal apparatus with lymphoma. Case presentation A 7-year-old girl with history of tearing, gradually prog...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

عنوان ژورنال:

دوره 17  شماره 

صفحات  -

تاریخ انتشار 2014