A case of diffuse large B-cell lymphoma transformed from immunoglobulin A-producing marginal zone B-cell lymphoma

We present a rare case of diffuse large B-cell lymphoma transformed from immunoglobulin (Ig) A-secreting marginal zone B-cell lymphoma. A 62-year-old woman was admitted to our hospital for examination of a disseminated pulmonary shadow. Gradual swelling of bilateral axilla and right inguinal lymph nodes were noted after admission. Histological examination of the lymph node biopsy specimen revealed the appearance of marginal zone B-cell lymphoma. The surface Ig of lymphoma cells was IgA-kappa, which coincided with the class of monoclonal Ig found in the patient's serum. The lymph node swelling and pulmonary shadow subsided, and the serum IgA level was normalized by 3 courses of systemic chemotherapy. However, after 4 courses of treatment, new tumor lesions at the right chest wall and left arm progressively became apparent. The biopsy specimen of the tumor showed a feature of diffuse large B-cell lymphoma. Despite intensive chemotherapy, the patient died of spreading tumor burden into the central nervous system.     

Clinical analysis of 3 cases with primary splenic diffuse large B-cell lymphoma

The definition of primary splenic lymphoma is controversial, but it has been reported to be a rare disease that comprises less than 1% of all malignant lymphomas. Three cases of primary splenic diffuse large B-cell lymphoma treated at our institution are described here. Median follow-up was 34.6 months (range 8.7～39.2) and median age at diagnosis was 72 years old (range 65～73). In all three cases, the diagnosis was definitively established not by splenectomy but by ultrasonically guided percutaneous splenic tissue core biopsy. Using the Hans classifier, one of the cases was subclassified as the germinal center B-cell like (GCB) subtype and two as non-GCB subtype. One case was CD5-positive diffuse large B-cell lymphoma. Two patients were in Ann Arbor stage II and one was in stage III. Using the International Prognostic Index, one was categorized as Low/intermediate risk, one as high/intermediate risk, and one as high risk. All patients underwent eight cycles of rituximab plus cyclophosphamide, doxorubicin, vincristine and prednisolone followed by irradiation therapy. These three patients attained complete response. Although the follow-up period to date has been short, all patients have maintained a complete response and are currently alive. To determine whether our management protocol is valid, further observations are needed.     

Primary bilateral adrenal lymphoma revealed by hemophagocytic syndrome

Primary adrenal lymphoma is rare. It is often bilateral and in most of the cases of B-cell type. The clinical features are various and not specific. We report a case of a 69-year-old woman who had a diffuse large B-cell lymphoma associated with hemophagocytic syndrome. The abdominal imaging reveals the existence of bilateral adrenal hypertrophy. A CT scan-guided biopsy concluded to a diffuse large B-cell lymphoma CD 20-positive associated EBV. The treatment consisted on “CHOP like” chemotherapy associated with rituximab. Primary adrenal lymphoma has a poor prognosis, even more poorly if associated with hemophagocytic syndrome.     

Aggressive transformation of a quiescent primary bone lymphoma simulating Paget's disease

Primary multifocal osseous lymphoma is a rare and poorly recognized entity. Here, we present an instructive case of a young man who, six years after a local contusion of the left ankle, developed a painful polylobulated large soft tissue mass. This mass turned out to have arisen from the transformation of a centro follicular non-Hodgkin's lymphoma into a diffuse large B-cell lymphoma involving the calcaneus, talus, cuboid and navicular bones. The diagnostic difficulties as well as the implications of this aggressive transformation are highlighted here.     

Recurrent Primary Cardiac Lymphoma on Aortic Valve Allograft: Implications for Therapy

Primary malignant cardiac lymphomas associated with grafts are extremely rare: to our knowledge, only 6 cases of prosthesis-associated B-cell lymphoma have been reported. Ours is the first report of recurrent diffuse large B-cell lymphoma associated with aortic valve allografts.We treated a 60-year-old man who presented in early 2007 with aortic valve endocarditis. He underwent aortic valve replacement with an allograft; the resected native valve showed active endocarditis without tumor. In January 2011, the patient underwent repeat aortic valve replacement because of symptomatic aortic regurgitation. The explanted valve specimen displayed diffuse large B-cell lymphoma. In September 2011, the patient presented with fever and a mass around the aortic valve. He died in January 2012. On autopsy, the explanted replacement valve displayed recurrent diffuse large B-cell lymphoma. The recurrent lymphoma on a new graft leads us to believe that this tumor is more aggressive than had been thought. We propose early systemic chemotherapy, in addition to tumor resection, for the possibility of a better prognosis. We discuss our patient''s case and review the relevant medical literature.     

Pancoast's syndrome in a patient with B-cell lymphoma diagnosed and confirmed with immunoglobulin gene rearrangement

Pancoast's syndrome due to malignant lymphoma is extremely rare. A case of diffuse large B-cell lymphoma presenting as Pancoast's syndrome is described. A 66-year-old man complained of pain and weakness of the right arm, and CXR revealed a right apical lung tumour. Histological findings were consistent with it being a diffuse large cell type lymphoma and Southern blot analysis revealed clonal rearrangement of the immunoglobulin heavy-chain JH. Thus, the tumour in this patient was diagnosed to be diffuse large B-cell lymphoma. Malignant lymphoma is an extremely rare cause of Pancoast's syndrome and only five cases have been described. This is the first reported case of Pancoast's syndrome caused by B-cell lymphoma, which was accurately diagnosed by analysis of gene rearrangement.     

Maladie des chaînes lourdes de type gamma associée à un lymphome B diffus à grandes cellules dans un contexte de myélodysplasie

IntroductionHeavy chain disease is a rare entity characterized by the production of incomplete immunoglobulin heavy chain without associated light chain. It is a B-cell lymphoproliferation, categorized according to the immunoglobulin involved. It is often associated with lymphomas but also with autoimmune diseases.ObservationWe report the case of a 70-year-old patient who presented a gamma-type heavy chain disease, associated with a diffuse large B-cell lymphoma in the context of myelodysplastic syndrome.ConclusionThis is the first case of diffuse large B-cell lymphoma associated gamma heavy chain disease described in the context of myelodysplastic syndrome.     

Hepatitis C virus infection and primary hepatic large B-cell lymphoma: a non-fortuitous association. Case report and review of literature

We report a case of hepatitis C virus infection in association with primary hepatic large B-cell non-Hodgkin's lymphoma. Primary hepatic non-Hodgkin's lymphoma is a rare disease. Association of hepatitis C virus infection with primary hepatic B-cell non-hodgkin's lymphoma is probably not fortuitous. Indeed, in case of primary hepatic non-hodgkin's lymphoma' patients are often hepatitis C virus positive. Moreover, several studies have reported a high prevalence of chronic hepatitis C virus infection among patients with B-cell non-Hodgkin's lymphoma whatever the localization of the lymphoma. A recent study found a high rate of remission of a splenic form of lymphoma after treatment of hepatitis C virus infection. Our case report confirms the hypothesis of a key role of hepatitis C virus in the pathogenesis of various forms of B-cell lymphoproliferative disorders and in particular in primary hepatic lymphoma.     

Secondary pancreatic involvement by a diffuse large B-cell lymphoma presenting as acute pancreatitis

Diffuse large B-cell lymphoma is the most common type of non-Hodgkin's lymphoma. More than 50% of patients have some site of extra-nodal involvement at diagnosis, including the gastrointestinal tract and bone marrow. However, a diffuse large B-cell lymphoma presenting as acute pancreatitis is rare. A 57-year-old female presented with abdominal pain and matted lymph nodes in her axilla. She was admitted with a diagnosis of acute pancreatitis. Abdominal computed tomography (CT) scan showed diffusely enlarged pancreas due to infiltrative neoplasm and peripancreatic lymphadenopathy. Biopsy of the axillary mass revealed a large B-cell lymphoma. The patient was classified as stage Ⅳ, based on the Ann Arbor Classification, and as having a high-risk lymphoma, based on the International Prognostic Index. She was started on chemotherapy with CHOP (cyclophosphamide, doxorubicin, vincristine and prednisone). Within a week after chemotherapy, the patient’s abdominal pain resolved. Follow-up CT scan of the abdomen revealed a marked decrease in the size of the pancreas and peripancreatic lymphadenopathy. A literature search revealed only seven cases of primary involvement of the pancreas in B-cell lymphoma presenting as acute pancreatitis. However, only one case of secondary pancreatic involvement by B-cell lymphoma presenting as acute pancreatitis has been published. Our case appears to be the second report of such a manifestation. Both cases responded well to chemotherapy.     

Diffuse large B-cell lymphoma presenting as a unilateral solitary round pulmonary hilar node infarction

Unilateral solitary pulmonary hilar node adenopathy is a rare presentation of diffuse large B-cell lymphoma. In this report, the authors present a case with a solitary pulmonary hilar lymph node infarction caused by diffuse large B-cell lymphoma. Enhanced CT examinations revealed a well-defined round mass with homogenous low attenuation in the left pulmonary hilum. Both radiological imaging and pathological examination can provide useful information for the interpretation of abnormalities and may enable the diagnosis of rare aetiologies.     

Low levels of monoclonal small B cells in the bone marrow of patients with diffuse large B-cell lymphoma of activated B-cell type but not of germinal center B-cell type

Background

Multiparameter flow cytometry allows the detection of minor monoclonal B-cell populations. Using this technique combined with morphology, we were struck by the presence of minor populations of small monoclonal B cells in bone marrows of patients with diffuse large B-cell lymphoma in routine diagnostic samples and performed a systematic retrospective study.

Design and Methods

Bone marrows of 165 patients with primary diffuse large B-cell lymphoma without histological evidence of concurrent non-Hodgkin’s lymphoma were studied by routine microscopy of trephines and smears, immunohistochemistry and multiparameter flow cytometry.

Results

Diffuse large B-cell lymphoma infiltration in marrows was documented in 11 of 165 patients. Morphological examination consistently revealed a higher tumor load than evidenced by flow cytometry. Of interest, only 3 of 119 patients with diffuse large B-cell lymphoma not otherwise specified, the largest subtype, showed marrow infiltration. By contrast, flow cytometry revealed a minor monoclonal B-cell population in 24 of 165 patients, none of whom showed diffuse large B-cell lymphoma infiltration by morphology. Of interest, morphological examination revealed the presence of small B cells in the marrows of those patients. Moreover, 11 of 39 (28.2%) of patients with diffuse large B-cell lymphoma not otherwise specified of ABC subtype and only 3 of 80 (3.7%) with the GCB subtype showed these monoclonal small B cells (P=0.0002). In addition 4 of 8 (50%), 4 of 15 (26.7%) and 2 of 3 (66.7%) patients with primary testicular, primary central nervous system and leg-type diffuse large B-cell lymphoma, respectively, showed monoclonal small B cells.

Conclusions

Bone marrow infiltration with diffuse large B-cell lymphoma in patients with diffuse large B-cell lymphoma not otherwise specified is rare at diagnosis. By contrast, a high number of diffuse large B-cell lymphoma not otherwise specified of the ABC subtype but not of GCB subtype is associated with monoclonal small B cells in the marrow. Whether these monoclonal small B cells are precursors of diffuse large B-cell lymphoma of the ABC type or arise in a common background that favors clonal B-cell expansion remains to be demonstrated.     

Asian variant of CD5+ intravascular large B-cell lymphoma with splenic infarction

A 57-year-old man was admitted with fever and epigastralgia, and presented with splenomegaly and pancytopenia. A CT scan revealed splenic infarctions. There were no lymphadenopathies, skin lesions, or neurological abnormalities. A splenectomy was performed. Bone marrow involvement with hemophagocytosis was noted. The diagnosis of Asian variant of intravascular diffuse large B-cell lymphoma was based on intravascular and sinusoidal distribution of large CD5+ B cells. The patient died of the disease 11 months after onset. To our knowledge, this is the first report of AIVL that presented with splenic infarction. This distinct lymphoma should be included in the differential diagnosis of splenic infarction.     

Primary T-cell lymphoma of the uterine corpus

Malignant lymphoma involving the genital tract is rare, and primary lymphoma of the uterus is even rarer. Immunohistochemically, most of the reported cases have been diffuse large cell lymphomas with a B-cell nature. Herein, we report an unusual case of uterine T-cell lymphoma in a 68-year-old woman. She suffered from fever, vaginal bleeding, and a huge mass in the lower abdomen. The initial clinical and histologic studies failed to indicate a cell type. We provide the clinical details of the case and discuss the diagnostic pitfalls.     

Refractory plasmablastic lymphoma—a review of treatment options beyond standard therapy

Plasmablastic lymphoma (PBL) is a rare distinct subtype of aggressive diffuse large B-cell lymphoma and a notoriously hard to treat entity with a dismal prognosis in both HIV-negative and HIV-positive patients. Clinicians often face the question of second or third line treatment. As the treatment options with novel agents in lymphomas are rapidly evolving, more and more options beyond standard chemotherapy are available. In connection with a review of treatment options with novel lymphoma agents, we present a case report of a patient with a complete remission after the administration of brentuximab vedotin and lenalidomide.     

Secondary intracardiac Burkitt-like lymphoma in the absence of HIV infection

Malignant lymphoma can involve the cardiac cavity or myocardium as a mass. Since clinical symptoms of its cardiac involvement are usually absent or non-specific, they may be undetected during life. Burkitt-like lymphoma (BLL) is a highly aggressive B-cell lymphoma with a high proliferative rate. Histopathological characteristics are considered borderline between those of classic Burkitt lymphoma and diffuse large B-cell lymphoma. Extensive cardiac involvement of BLL is rare and poorly documented in the literature. We report the case of a child with BLL, presenting with extensive infiltration of the heart in the absence of HIV infection, and with right-sided heart failure and positional dyspnoea as the major clinical problems. We highlight the challenges for diagnosis and adequate treatment in poor settings like ours.     

Spinal cord compression by primary epidural non-Hodgkin lymphoma

A 63-year-old man was admitted because of sudden paraparesis. Myelography revealed a complete block at the level of T-3. The spinal epidural tumor was histologically diagnosed as malignant lymphoma, diffuse large cell type and immunohistochemically B-cell type. A thorough work-up for metastasis including MRI was negative. He was postoperatively treated with combination chemotherapies (VEPA), followed by irradiation to the region. The residual tumor disappeared on chest-CT and the patient's neurological status rapidly improved. He is still in remission for 18 months and has full performance status. This case is a very rare lymphoma originated from spinal-epidural space.     

Hepatic tumor rupture following effectual treatment with irinotecan in a patient with highly refractory malignant lymphoma

We describe a patient with highly refractory malignant lymphoma who died of hepatic tumor rupture following treatment with irinotecan (CPT-11). This 60-year-old man with non-Hodgkin's lymphoma (diffuse large B-cell lymphoma) demonstrated disease recurrence in the liver and the vertebrae following high-dose chemotherapy and autologous hematopoietic stem cell transfusion. He was treated with CPT-11 at a dose of one third of the conventional dose used for non-Hodgkin's lymphoma in Japan. The tumor in the liver markedly decreased in size but then ruptured. Although pathologic hepatic tumor rupture is a rare complication in patients with malignant lymphoma of the liver, this case demonstrates that hepatic tumor rupture may occur in refractory malignant lymphomas that reveal extensive degradation by this new, effective salvage therapy.     

Extranodal multifocal Rosai–Dorfman disease: response to 2-chlorodeoxyadenosine treatment

Rosai–Dorfman disease (RDD) or “sinus histiocytosis with massive lymphadenopathy” is a rare lymphoproliferative disorder of unknown etiology. The disease usually presents with painless lymphadenopathy with occasional extranodal involvement in various organs. We report a case of a 36-year-old man with a history of non-Hodgkin lymphoma (NHL), who recently presented with inguinal lymphadenopathy. Following the diagnosis of RDD on lymph node biopsy, he developed symptoms of spinal cord compression due to a mass lesion discovered at T6-7 vertebral level. 18F-Fluorodeoxyglucose (18FDG) positron emission tomography (PET-CT) revealed extensive disease with lung, renal and bone involvement. The patient received a short course of steroid therapy for cord compression findings and 2-chlorodeoxyadenosine (2-CdA) treatment was initiated for long-term disease control. He had a dramatic sustained response to treatment with six courses of 2-CdA. These results suggest that 2-CdA can be an effective treatment of choice and positron emission tomography with 18FDG can be used for determining the extent of disease and for follow-up in RDD.     

Diffuse large B-cell lymphoma complicated with autoimmune thrombocytopenia

A patient was diagnosed with autoimmune thrombocytopenia and concurrent diffuse large B-cell lymphoma (DLBCL). Both autoimmune thrombocytopenia and DLBCL were successfully treated using chemotherapy, while steroid therapy, rituximab monotherapy, and high-dose parenteral glucocorticoid therapy combined with high-dose intravenous immunoglobulin failed to improve thrombocytopenia. A greater understanding of the pathogenesis and treatment options can only be definitively clarified by analyses of settings such as the present case, as autoimmune thrombocytopenia is uncommon in patients with malignant lymphoma and the etiology and optimal treatment remain unclear.     

Primary splenic CD8-positive diffuse large B-cell lymphoma

A 71-year-old woman was admitted for further examination of an increased serum LDH level. Abdominal ultrasonography and CT scan showed a large tumor in her spleen. Because malignant lymphoma was suspected, the spleen was removed for diagnosis and treatment planning. The histopathological and immunohistochemical features of the tumor indicated diffuse large B-cell lymphoma (DLBL). The flow-cytometric immunophenotype of the lymphoma cells was CD2-, CD3-, CD4-, CD5-, CD8+, CD10+, CD19+, CD20+, CD23-, kappa+, lambda-, CD25+, and CD56-. From these findings, the patient was diagnosed as having CD8+ DLBL. To our knowledge, this is the first reported case of primary splenic CD8-positive DLBL.