1/6. Non-fatal haemophagocytic syndrome in an elderly patient.A frail 78-year-old man presented with lethargy, fever, splenomegaly and pancytopenia. bone marrow aspirate showed marked haemophagocytosis. A diagnosis of haemophagocytic syndrome secondary to diffuse splenic large B-cell lymphoma was eventually made. Treatment with laparascopic splenectomy was successful. Secondary haemophagocytic syndrome is a rare complication of many common conditions, and is fatal if untreated. A brief literature review is included.- - - - - - - - - - ranking = 1keywords = lymphoma (Clic here for more details about this article) |
2/6. Aggressive Lennert's lymphoma: report of three cases in comparison to non-aggressive Lennert's lymphoma.The present article describes three cases of Lennert's lymphoma exhibiting aggressive clinical courses. These cases were accompanied by disseminated intravascular coagulation (DIC) or hemophagocytic syndrome (HPS). These cases were compared to non-aggressive type of Lennert's lymphoma. Of the three cases, two demonstrated involvement of the liver and the other possessed bone marrow involvement. In one patient, while a lymph node biopsy revealed Lennert's lymphoma histologically, a liver biopsy obtained 2 months later revealed a high-grade large cell cytotoxic T-cell lymphoma. Two of these cases showed HPS and the other exhibited DIC. All patients died within 1 year of diagnosis, with the shortest survival period being 1.5 months. Immunohistochemically, lymphoma cells were CD8 , CD4-, granzyme B , and T-cell intracellular antigen-1 (TIA-1) , showing a cytotoxic T-cell phenotype. Two cases demonstrated positive reactivity for Epstein-Barr virus in lymphoma cells by in situ hybridization. These cases were compared with eight cases of non-aggressive Lennert's lymphoma. In comparison to non-aggressive disease, these three cases displayed a higher percentage of Ki-67-positive cells. In conclusion it was found that a subset of Lennert's lymphoma cases share common features with high-grade cytotoxic T-cell lymphoma, indicating that Lennert's lymphoma may be part of the spectrum of cytotoxic T-cell lymphoma.- - - - - - - - - - ranking = 19keywords = lymphoma (Clic here for more details about this article) |
3/6. Haemophagocytic syndrome associated with hepatitis-B virus infection responding to etoposide.Haemophagocytic syndrome (HPS) secondary to viral infections usually has a variable course and can be life-threatening. We report a 53-year-old male patient who presented with fever, hepatosplenomegaly and pancytopenia. He had deranged liver function, abnormal clotting and markedly elevated serum ferritin. bone marrow biopsy showed prominent haemophagocytosis. The patient was investigated thoroughly and found to have evidence of chronic hepatitis b-virus (HBV) infection by serological tests and liver biopsy. Other conditions associated with HPS such as lymphoma, malignancy and other viral or bacterial infections were not present. The patient did not respond to steroids, intravenous immunoglobulins or cyclosporin but responded to etoposide and became apyrexial. He also became HBV negative on lamivudine. The patient died of infection later on but there was no evidence of recurrence of HPS. To the best of our knowledge this is the first case report of HPS associated with isolated HBV infection.- - - - - - - - - - ranking = 1keywords = lymphoma (Clic here for more details about this article) |
4/6. Fatal HHV-8-associated hemophagocytic syndrome in an hiv-negative immunocompetent patient with plasmablastic variant of multicentric Castleman disease (plasmablastic microlymphoma).Virus-associated hemophagocytic syndrome (VAHS) triggered by HHV-8 is extremely rare and has been reported only in 9 immunocompromised patients. We report the first case of HHV-8-associated VAHS in an hiv-negative, immunocompetent patient with plasmablastic variant (plasmablastic microlymphoma) of multicentric Castleman disease (MCD). This 61-year-old man presented with fever, cough, and bilateral inguinal lymphadenopathy. biopsy of the right inguinal lymph node revealed plasmablastic MCD with nodular aggregates of plasmablasts expressing IgM, MUM1, HHV-8 latency-associated nuclear antigen, and viral interleukin-6. These plasmablasts were monotypic for Iglambda light chain expression but not Igkappa. All the B-cell clonality assays, including IgH-FR2, IgH-FR3, DH-JH, Igkappa, and Iglambda PCR, showed a polyclonal pattern. His serum human interleukin-6 level was markedly elevated and was negative for EBV acute infection/reactivation. The marrow aspirate showed florid hemophagocytosis. His disease progressed rapidly to multisystemic illness, and he died of acute respiratory failure in 1 month. Our case showed that HHV-8 might trigger VAHS in an immunocompetent patient with plasmablastic MCD. We speculated that our patient developed VAHS under the cytokine storm associated with the proliferating HHV-8-infected plasmablasts, similar to the EBV-triggered VAHS in patients with EBV-associated T-cell lymphoma.- - - - - - - - - - ranking = 6keywords = lymphoma (Clic here for more details about this article) |
5/6. Subcutaneous panniculitis-like T-cell lymphoma: successful initial treatment with prednisolone and cyclosporin A.A case of subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is reported. A 27-year-old man presented with fever and abdominal swelling. His laboratory examination revealed pancytopenia and liver dysfunction. The diagnosis of SPTCL was made by biopsy based on thickened subcutaneous tissue. In addition, bone marrow specimen showed a hemophagocytosis syndrome (HPS). methylprednisolone pulse therapy was initiated followed by prednisolone (60 mg/day) and cyclosporin A (150 mg/day). He responded to the treatment and remained asymptomatic for at least for 6 months. Our results suggest that a trial of cyclosporin A is warranted in patients with SPTCL complicated by HPS.- - - - - - - - - - ranking = 5keywords = lymphoma (Clic here for more details about this article) |
6/6. Subcutaneous panniculitis-like T-cell lymphoma with hemophagocytic syndrome successfully treated with cyclosporin A.A 17-year-old girl previously in good health presented with a 2-month history of recurrent, high-grade fever; general fatigue; anorexia; a 10-kg weight loss; and multiple, painful, reddish skin lesions on the lower abdomen. Some lesions were ulcerated, with an oily yellowish brown discharge. A systemic review was unremarkable other than bleeding from the nose. Her medical and family histories were unremarkable. On examination, the patient was pale, jaundiced, and febrile (temperature of 39 degrees C). She had enlarged lymph nodes in the axillary and inguinal areas. There was moderate hepatosplenomegaly. Local skin examination revealed multiple erythematous, tender, and firm subcutaneous nodules of variable size (1-2 cm) on the lower abdomen. Some nodules were ulcerated, with oily yellowish brown discharge and overlying ecchymosis (Figures 1 and 2). Mucous membranes were free of lesions. Laboratory investigations showed pancytopenia, an elevated erythrocyte sedimentation rate (>80 mm/h), normal renal function tests, abnormal hepatic function tests (alanine aminotransferase 172 U/L, aspartate aminotransferase 229 U/L, alkaline phosphatase 725 U/L, and total bilirubin 100 mmol/L [normal range 0-18 mmol/L]), conjugated bilirubin 45 mmol/L (normal range 0-5 mmol/L), and high triglycerides 855 mg/dL (normal range 20-200 mg/dL). Prolonged prothrombin time, 26 seconds (normal range 13-16 seconds); prolonged activated partial thromboplastin time, 61 seconds (normal range 26-38 seconds); positive disseminated intravascular coagulation studies evidenced by low fibrinogen, 74 mg/dL (normal range 160-350 mg/dL); and positive fibrinogen degradation products were also noted. Throat, midstream urine, and blood culture results were negative. serologic tests for syphilis, hiv, and hepatitis b and C viruses were negative. Epstein-Barr virus and cytomegalovirus serologic values revealed evidence of past infection. tuberculin and Coombs tests were negative. The alpha1-antitrypsin level was normal. Antinuclear and anti-smith antibodies, rheumatoid factor, and cryoglobulins were negative. CT showed enlarged lymph nodes in the axillary and inguinal areas, bilateral small pleural effusion, moderate hepatosplenomegaly, severe fatty infiltration of the liver, and thickening of lower abdominal subcutaneous tissue. A liver biopsy showed steatohepatitis. bone marrow aspirate and trephine were normal. A deep punch biopsy of a nodule from the right lower abdomen revealed lobular panniculitis with atypical lymphocytes and large macrophages with cytophagocytosis ("beanbag" cells) (Figures 3 and 4). immunohistochemistry showed that these atypical cells were positive for CD3, CD8, granzyme B, and perforin, and negative for CD56. T-cell gene rearrangement studies on skin lesions revealed a monoclonal T-cell receptor (gamma-chain) gene rearrangement, supporting the diagnosis of subcutaneous panniculitis-like T-cell lymphoma. On presentation, the initial treatment included 6 U of fresh frozen plasma, 2 U of packed red blood cells, and 2 g IV fibrinogen for 3 consecutive days. The patient was started on prednisolone 60 mg orally once daily and cyclosporine A 5 mg/kg/d orally in two divided doses. The fever and other systemic symptoms and skin lesions resolved within 2 weeks after the treatment. The prednisolone dose was tapered gradually, and a maintenance dose of cyclosporine A was continued. The patient's condition remained in remission at 12-month follow-up; there was no evidence of clinical relapse.- - - - - - - - - - ranking = 5keywords = lymphoma (Clic here for more details about this article) |