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Objective:To investigate clinicopathological features of 4 cases of intravascular large B-cell lymphoma (IVLBCL) .Methods:Clinical and pathological data were collected from 4 patients with histopathologically confirmed IVLBCL in Department of Dermatology, Peking Union Medical College Hospital from January 2020 to November 2020, and retrospectively analyzed.Results:The 4 patients were aged 57 - 76 years, including 2 males and 2 females. Of the 4 patients, all had neurological symptoms, 3 had fever, 3 exhibited impaired exercise tolerance and suffocation, and 3 exhibited pitting edema of the body. Case 1 presented with a cherry hemangioma-like papule measuring 0.2 cm in diameter on the back, and case 2 with telangiectasia on the left breast and upper abdomen. Six skin samples were taken from the 4 patients for histopathological and immunohistochemical studies, and tumor cells were found in 1 cherry hemangioma-like lesion and 1 lesion of telangiectasia, as well as in 2 of 4 normal skin samples. Histopathological findings mainly were dilated dermal blood vessels filled with large atypical mononuclear cells, and the atypical mononuclear cells were positive for CD20 immunohistochemically.Conclusion:For those patients with suspected IVLBCL, hemangioma-like and telangiectasia lesions tend to show characteristic histopathological and immunohistochemical findings, and a biopsy of normal skin can facilitate early diagnosis of IVLBCL.
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Objective:To investigate the diagnosis and treatment of intravascular large B-cell lymphoma (IVLBCL).Methods:The clinical data of 1 patient with adrenal IVLBCL in Zhongda Hospital Southeast University in May 2020 were retrospectively analyzed, and the relevant literature was reviewed.Results:The patient was an elderly male with recurrent fever of unknown cause at initial stage, and was finally diagnosed as adrenal IVLBCL based on the results of laboratory, imaging and adrenal biopsy at different stages. After multiple courses of R-COP in combination with Bruton tyrosine kinase (BTK) inhibitor, the patient achieved complete remission.Conclusions:IVLBCL is rare and it lacks specific clinical symptoms. PET-CT and pathological biopsy can help in the diagnosis of it. R-COP combined with BTK inhibitor is effective in the treatment of biphenotype IVLBCL.
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Abstract Intravascular large B-cell lymphoma is a rare, non-mass-forming, extranodal large B-cell lymphoma subtype characterized by the presence of tumor cells in the lumens of vessels. It is divided into two major types: classical and Asian. Patients presenting only with skin involvement are mostly female, at a younger age than classical intravascular large B-cell lymphoma patients, and have a better prognosis. Since the diagnosis of cases with isolated skin involvement is difficult, keeping this entity in mind, performing a careful microscopic examination, and applying new, effective treatment regimens will make it possible to achieve better clinical outcomes in these cases.
Subject(s)
Humans , Female , Skin Neoplasms/diagnosis , Panniculitis/diagnosis , Lymphoma, Large B-Cell, Diffuse/diagnosis , Lymphoma, Large B-Cell, Diffuse/drug therapy , Prognosis , SkinABSTRACT
Resumen El linfoma intravascular de células B grandes es una rara variante del linfoma no Hodgkin que se caracteriza por proliferación clonal de células linfoides neoplásicas que crecen dentro de la luz de la microvasculatura. La diversidad de su presentación clínica dada su posibilidad de aparición en cualquier órgano, a menudo lo convierte en un diagnóstico difícil. El espectro clínico es heterogéneo dominado por manifestaciones neurológicas y puede pasarse por alto o diagnosticarse erróneamente. El diagnóstico sigue siendo un reto, ya que no existen hallazgos patognomónicos de neuroimágenes y aún no se ha establecido un tratamiento óptimo. Presentamos el caso de una mujer de 38 años con linfoma intravascular de células B grandes con afección cerebral.
Abstract Intravascular large B-cell lymphoma is a rare variant of non-Hodgkin lym- phoma characterized by clonal proliferation of neoplastic lymphoid cells that grow within lumen of the microvasculature. The diversity of the clinical presentation due to the possibility of its appearance in any organ often makes it a difficult diagnosis. Clinical spectrum is heterogeneous, dominated by neurological manifestations can be overlooked or misdiagnosed. Diagnosis remains a challenge, since there are no pathognomonic neuroimaging findings and optimal treatment has not yet been established. We present a 38-year-old female patient with intravascular large B-cell lymphoma with cerebral involvement.
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Introduction: Intravascular large B-cell lymphoma (IVLBCL) is a rare disease entity of non-Hodgkin lymphoma. Patients with IVLBCL frequently have neurological symptoms associated with cerebrovascular infarction or central nervous system involvement of malignant lymphoma. Case: A 67-year-old man consulted the Department of Hematology at our hospital because of fever of unknown origin, anemia and increased serum lactate dehydrogenase. Although IVLBCL was strongly suspected, no lymphoma cells were found by multiple bone marrow aspirations and skin biopsies. Two months later, he developed hyperactive delirium, which was difficult to manage using antipsychotic agents. Brain MRI revealed multiple hyper-intense infarct-like lesions on diffusion-weighted images. After assessment of bone marrow aspiration and skin biopsies, he was administered an enough dose of prednisolone to manage malignant lymphoma. Hyperactive delirium rapidly improved. Discussion: In patients with IVLBCL, corticosteroids may be useful to manage hyperactive delirium due to cerebrovascular infarction or central nervous system involvement of IVLBCL.
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BACKGROUND: Intravascular large B-cell lymphoma (IVLBCL) is a rare type of extranodal lymphoma with growth mainly in the lumina of vessels. We studied a small series of IVLBCL and focused on its central nervous system (CNS) involvement. METHODS: Searching the medical records of Northwestern Memorial Hospital, we identified five cases of IVLBCL from January 2007 to January 2015. Clinical information, hematoxylin and eosin stained histologic slides and immunohistochemistry studies were reviewed for all cases. Polymerase chain reaction (PCR) analysis for the immunoglobulin (Ig) heavy and light chain gene rearrangement was performed on all five cases. RESULTS: Three of the five cases of IVLBCL were autopsies. Patients’ age ranged from 56 to 84. CNS involvement was present in two cases—in both patients, the CNS involvement showed an extravascular pattern with confluent sheet-like formation. PCR analysis confirmed that in one case the systemic intravascular and CNS extravascular components were clonally identical. CONCLUSIONS: In a small case series of IVLBCL, we observed that CNS involvement by IVLBCL often has an extravascular morphology, but is clonally identical to the intravascular counterpart by PCR analysis. As IVLBCL can have a rapidly progressing poor outcome, it should be kept in the differential diagnoses for patients presenting with lymphoma of the CNS. The presence of extravascular growth patterns in the CNS should not exclude IVLBCL as a diagnosis.
Subject(s)
Humans , Autopsy , B-Lymphocytes , Central Nervous System , Diagnosis , Diagnosis, Differential , Eosine Yellowish-(YS) , Gene Rearrangement , Hematoxylin , Immunoglobulins , Immunohistochemistry , Lymphoma , Lymphoma, B-Cell , Medical Records , Polymerase Chain ReactionABSTRACT
A 68-year-old retired nurse, who was a known hypertensive on medication, presented with prolonged fever of 2-month duration without any clinical evidence of infection. On examination she had altered mental status. She also had other nonspecific complaints such as sleep disturbances, loss of weight, etc. On investigation, she was found to have anemia, thrombocytopenia, raised erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and lactate dehydrogenase (LDH) values. She also had electrolyte imbalance. Radiological evaluation of brain showed mass lesion in the sella turcica, suggestive of pituitary adenoma. Biochemical evaluation showed hypopituitarism. Trans-sphenoidal biopsy was done. Based on histopathological and immunohistochemical findings a diagnosis of intravascular large B-cell lymphoma (IVLBCL) of pituitary was made. Our patient's condition deteriorated rapidly and she succumbed to her illness before therapy could be initiated. We are reporting this case because of the rare subtype of large B-cell lymphoma presenting at an extremely unusual primary site.
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Intravascular large B cell lymphoma is a rare subtype of diffuse large B cell lymphoma. Abnormal proliferation of intravascular lymphoma cells is its pathological feature with aggressive clinical behavior such as easy invasive feature, rapid disease progression, poor prognosis and low survival rate. We review the progress in recent research on classification, diagnosis, treatment and prognosis of intravascular large B cell lymphoma.
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Intravascular large B-cell lymphoma (IVLBCL) is a subtype of diffuse large cell lymphoma, characterized by proliferation of lymphoid cells in the intravascular space of various organs without causing a mass effect. Although 18F-FDG PET is a powerful imaging tool in lymphoma, the usefulness of 18F-FDG PET in the assessment of IVLBCL is still controversial. 99mTc-MIBI, a tumor imaging radiopharmaceutical with a different mechanism from that of 18F-FDG, has been reported to be also effective in lymphoma. However, there is nearly no report on the efficacy of 99mTc-MIBI in the assessment of IVLBCL. We present one case of IVLBCL that showed 99mTc-MIBI accumulation in the involved bone marrow as an incidental finding, which was discrepant from that of 18F-FDG PET.
Subject(s)
B-Lymphocytes , Bone Marrow , Fluorodeoxyglucose F18 , Incidental Findings , Lymphocytes , Lymphoma , Lymphoma, B-Cell , Lymphoma, Large B-Cell, DiffuseABSTRACT
Intravascular large B-cell lymphoma is rare and generally fatal. It is defined pathologically by neoplastic proliferation of lymphoid cells within the lumens of capillaries, small veins, and arteries with little or no other parenchymal involvement. The diagnosis can be delayed because of the rarity of the disease and the difficulty of detection in imaging studies, and a suspicious clinical observation is warranted to make the correct diagnosis. Early diagnosis is important because delayed treatment could result in a fatal outcome. We have encountered a case of intravascular large B-cell lymphoma involving only the bone marrow. An early diagnosis was made and the patient was treated with combination chemotherapy and rituximab targeting CD20. The patient went into complete remission after the third cycle of chemotherapy and maintained a disease free state up to 6 months.
Subject(s)
Humans , Arteries , B-Lymphocytes , Bone Marrow , Capillaries , Diagnosis , Drug Therapy , Drug Therapy, Combination , Early Diagnosis , Fatal Outcome , Lymphocytes , Lymphoma, B-Cell , Veins , RituximabABSTRACT
Intravascular large B-cell lymphoma (IVLBL) is a rare neoplasm characterized by proliferation of lymphoma cells within the lumina of small vessels. Neurological and skin involvements usually predominate. We would describe a 78-year-old woman presented with fever, multiple erythematous skin lesions, and language disturbance. The skin biopsy of breast revealed IVLBL and malignant cells were also seen in the bone marrow. Shortly after completion of six cycles of CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisolone) with clinical response, intracranial relapse with multiple brain masses occurred. The palliative whole brain radiation therapy was given and intensive chemotherapy should be investigated in the case presented here.
Subject(s)
Aged , Female , Humans , B-Lymphocytes , Biopsy , Bone Marrow , Brain , Breast , Doxorubicin , Drug Therapy , Fever , Lymphoma , Lymphoma, B-Cell , Recurrence , Skin , VincristineABSTRACT
Intravascular large B-cell lymphoma(IVLBCL) is a rare form of diffuse LBCL characterized by preferential intravascular growth of malignant lymphocytes,aggressive behavior,and an often fatal course.It displays some differences in clinical presentation among diverse geographical areas.The immunophenotype plays a critical role in diagnosis and differential diagnosis.Recent therapeutic approaches,such as the rituximab plus CHOP regimen,could have a positive impact in IVLBCL patients.The use of high-dose chemotherapy supported by autologous stem-cell transplantation may improve current outcomes.