Primary Plasma Cell Leukaemia in a Young HIV Infected Male, Associated with Epstein-barr Virus (original) (raw)
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Primary plasma cell leukemia in 32 years old male-A case report
2015
Plasma cell leukemia (PCL) is a rare disease and is the least common variant of multiple myeloma accounting for 2-3% of all plasma cell dyscrasias. Histogenetically, plasma cell leukemia is derived from terminally differentiated B cells. It is diagnosed by presence of absolute plasma cell count >2000 per cm or >20% circulating plasma cells. Here we report a case of plasma cell leukemia, who presented with easy fatigability, weakness and high grade fever since 1 month. Hematological investigation revealed leukocytosis with plasmacytosis (7420/ mm 3). On bone marrow examination, >45% plasma blasts were seen. Biochemical analysis showed high LDH level (4236 U/L) and serum calcium level was also raised (12.3 mg/dl). Final diagnosis of plasma cell leukemia was made. As PCL is rare disease and it is even rarer to find them in a 32 years old. Here we are able to find and document the typical features of PCL.
Case Reports in Hematology, 2019
EBV (Epstein–Barr virus) viremia causes immune dysregulation through various mechanisms, and we are understanding more that mutations in B, T, and NK (natural killer) cell signaling pathways allow EBV complications such as HLH (hemophagocytic lymphohistiocytosis) and lymphomas to arise. Here, we report a 20-year-old previously healthy, HIV- (human immunodeficiency virus-) negative male who presented with fevers, sore throat, and lymphadenopathy (LAD). He was found to have EBV viremia, pancytopenia, and elevated LFTs (liver function tests) suspicious for HLH. Bone marrow biopsy and elevated IL-2 (interleukin) receptor confirmed this diagnosis. Additionally, gastric biopsy confirmed diagnosis of plasmablastic lymphoma (PBL), a rare, aggressive HIV- and EBV-associated lymphoma. Both bone marrow and gastric biopsy showed evidence of EBV. Patients with EBV complications should have a rigorous workup to characterize the full extent of immune dysregulation including genetic testing at a hi...
International Journal of Hematology, 2013
Plasma cell leukaemia (PCL) is a rare condition with high mortality. HIV-positive patients have a propensity to develop malignancy; however, the occurrence of PCL with HIV infection in South Africa has not been documented. We describe patients with PCL in Universitas Hospital in Bloemfontein, South Africa, and report two new cases of HIV infection concurrent with PCL. A retrospective case series of PCL patients (2006)(2007)(2008)(2009)(2010)(2011)(2012) seen at our Clinical Haematology unit is reported. Patient files were used to obtain information. The median age of patients (n = 9) was 51 years, and 66.7 % of cases were of African ethnicity. The condition was equally distributed between genders. Two patients were HIV positive. Both received combination antiretroviral therapy. The diagnosis of PCL was usually made as an incidental finding, subsequently confirmed on bone marrow aspirate and trephine. Deranged haematological and biochemical parameters, including severe anaemia, hypoalbuminaemia, and hyper-cellular bone marrow, were observed. Only one patient improved markedly on treatment, and remains alive at the time of writing. PCL shows poor response to treatment and predominates among Africans. The small sample size made it difficult to determine whether co-infection with HIV was a coincidental finding or the two diseases are pathophysiologically linked.
Primary plasma cell leukaemia in a 22-year-old woman: A case report
African Journal of Laboratory Medicine, 2015
Introduction: Primary plasma cell leukaemia is a rare and highly aggressive disease that is commonly diagnosed a decade earlier than multiple myeloma, at a median age of 55 years. However, it has also been described in younger patients, as documented in this case report. It often presents with hepatosplenomegaly and lymphadenopathy, whilst the presence of bony lesions are less-commonly seen when compared to multiple myeloma.Case presentation: This report describes the case of a young woman who presented with symptoms of anaemia and a history of menorrhagia. On further careful examination, she was found to have additional signs and symptoms and was later diagnosed with primary plasma cell leukaemia.Management and outcome: On admission, the patient received supportive care measures, including blood products. At diagnosis, a specific chemotherapy regimen was commenced; however, this failed to induce remission. The decision to continue with supportive care only was made and the patient ...
Secondary Plasma Cell Leukaemia
2020
Plasma cell leukaemia is a clinical condition in which plasma cells circulating in the peripheral blood constitute >20% of white blood cells (WBC) and there is evidence of plasma cell monoclonality. It is important to be diagnosed early for better treatment outcome. Although it is a rare disease, cases have been reported from Pakistan and other countries (including our neighbouring countries), hence making this case report. After taking history of present and past ailments, physical examination was carried out. Blood and bone marrow sampling were done after taking informed written consent from the patient. Blood samples were obtained in plain bottle, anticoagulated bottle and bone marrow was obtained from posterior iliac spine under 2% lignocaine. Plasma cell leukaemia is a rare and aggressive disease, difficult to diagnose and treat, requires early recognition and therapeutic intervention.
Primary plasma cell leukaemia: a report of 18 cases
Leukemia Research, 2001
Primary plasma cell leukaemia (P-PCL) is a variant of multiple myeloma (MM) first diagnosed in the leukemic phase, with \2000/mm 3 circulating plasma cells (PCs) and plasmacytosis \ 20% of the white cell count. We investigated the clinical characteristics, therapy, immunophenotype and prognosis factors of 18 patients. Common features at diagnosis were asthenia (seven patients), renal insufficiency (ten patients), bone pain (seven patients), splenomegaly or hepatomegaly (five patients). Hypercalcemia was present at diagnosis in seven patients and was the most potent poor prognosis factor (PB 0.05). Most patients (16 out of 18) were treated with an anthracyclin containing regiment; complete remission was attained in one patient and partial remission in 11 patients while six patients had no response. The median survival time from diagnosis was 7 months (2 -12, 95% confidence interval), but response to treatment had favorable predictive value (PB 0.05). The PCs were usually positive for mature B-cell markers (PCA-1, CD38). They expressed integrins which may increase their binding to endothelial cells and thus participate in PCL physiopathology by favoring plasmocyte extramedullary spread.
Indian Journal of Hematology and Blood Transfusion, 2012
Plasma cell leukemia (PCL) represents a rare and aggressive form of plasma cell dyscrasia which can be primary (pPCL) or secondary (sPCL). It is diagnosed based on absolute plasma cell count of more than 2.0 9 10 9 /l or a relative proportion of greater than 20% of the peripheral blood leukocyte count. Although pPCL and sPCL share several clinical features, important differences exist. Patients with pPCL are younger; often have extra osseous organ involvement (liver, spleen and other extramedullary sites), increased frequency of renal failure, fast declining performance status and rapid progression to the terminal stage. Patients with sPCL have advanced bone disease. Presented in this article is India data of a short series of five cases of PCL diagnosed at a tertiary care centre from south India over last 5 years. All cases were de novo and had varied spectrum of presentation and so were not suspected to be plasma cell dyscrasia clinically. Detailed hemato-pathological evaluation clinched the diagnosis in all the cases.
HIV-associated plasmablastic lymphoma: Lessons learned from 112 published cases
American Journal of Hematology, 2008
Plasmablastic lymphoma (PBL) is a distinct subtype of non-Hodgkin B-cell lymphoma, originally described with a strong predilection to the oral cavity of human immunodeficiency virus (HIV)-infected individuals. Data regarding patient age and gender, HIV status, initiation of and response to highly active antiretroviral therapy (HAART), tumor extent, pathology, treatment, and outcome were extracted from 112 cases of PBL identified in the literature. The median age at presentation was 38 years with a male predominance of 7:1, and the median CD4+ count was 178 cells/mm3. PBL presented on average 5 years after diagnosis of HIV. Common primary sites of presentation included the oral cavity, gastrointestinal tract, and lymph nodes. Most cases presented with either stage I or stage IV disease. There was a variable expression of B-cell markers in tumor cells, but plasma cell markers were expressed in all cases. EBV was detected in 74%. Chemotherapy was used to treat 55% patients and was combined with radiotherapy in 21% cases. Complete response was obtained in 66% of treated cases; the majority of these responses were seen after CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone). The refractory/relapsed disease rate was 54%. Death occurred in 53% of patients, with a median overall survival of 15 months. Sex, CD4+ count, viral load, clinical stage, EBV status, primary site of involvement, and use of CHOP failed to show an association with survival. PBL is an aggressive B-cell lymphoma that presents in both oral and extra-oral sites of chronically HIV-infected immunosuppressed young men. Am. J. Hematol., 2008. © 2008 Wiley-Liss, Inc.
South Asian Journal of Cancer
Background Plasmablastic lymphoma (PBL) is a rare aggressive B cell lymphoma that is commonly encountered in patients with human immunodeficiency virus (HIV)/acquired immunodeficiency syndrome (AIDS). In this case series, we describe the clinicopathological features of cases of PBL seen at a tertiary care center in South India. Materials and Methods Medical records of patients diagnosed with PBL between January 2009 and November 2017 were reviewed. PBL was defined as per the World Health Organization 2016 classification for hematopoietic and lymphoid neoplasms. The slides were reviewed with hematoxylin and eosin along with immunohistochemistry (IHC) including CD45, CD20, PAX5, CD79a, CD3, CD5, CD138, MUMI, EMA, ALK, and Ki67. Epstein-Barr virus (EBV) association was documented by rapid in situ hybridization (RISH) studies wherever possible. The demographic data, clinical presentation, treatment details, and outcomes are elaborated using descriptive statistics. Results During the stu...