Intravascular large B-cell lymphoma (IVLBCL): a clinicopathologic study of 96 cases with special reference to the immunophenotypic heterogeneity of CD5

Murase, Takuhei; Yamaguchi, Motoko; Suzuki, Ritsuro; Okamoto, Masanori; Sato, Yumiko; Tamaru, Jun Ichi; Kojima, Masaru; Miura, Ikuo; Mori, Naoyoshi; Yoshino, Tadashi; Nakamura, Shigeo

doi:10.1182/blood-2006-01-021253

Cited by 379 publications

(437 citation statements)

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“…Prognosis is uniformly poor with better outcomes observed with the ''cutaneous variant,'' which is associated with skin lesions in the absence of systemic involvement [6][7][8][9]. An ''Asian variant'' of the disease mostly reported in the Japanese literature differs from the presentation of IVL in the Western literature as it is associated with a hemophagocytic syndrome, bone marrow involvement, hepatosplenomegaly, and thrombocytopenia, while cutaneous and CNS lesions are uncommon [15,16]. IVL is considered a disseminated disease and treatment with combination chemotherapy including anthracyclines is recommended along with inclusion of methotrexate or cytarabine when there is CNS involvement [7].…”

Section: Commentarymentioning

confidence: 99%

A 69‐year‐old male presenting with hypotension and anasarca

et al. 2007

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A 69-year-old Caucasian male with a past medical history remarkable for hypertension and testicular nonHodgkin's lymphoma (NHL) presented to the emergency department with a 1-week history of fatigue and new onset lower extremity edema. For the past 8 months, the patient was having a nonproductive cough. Work up by his primary care physician was negative, and the patient was switched from enalapril to losartan 1 week before presentation. The cough resolved, however, 2 days later the patient began to experience fatigue and lightheadedness that persisted despite decreasing the dose of losartan. He continued to develop lower extremity edema and was again seen by his primary care physician who noted the patient to be hypotensive. He was referred to the emergency department for evaluation.In a 69-year-old male presenting with fatigue, lower extremity edema, and hypotension, our first concern was for a cardiac process such as heart failure. The differential for his hypotension also included sepsis, adrenal insufficiency, hemorrhage, or possibly an adverse drug reaction. We suspected that his cough was secondary to the enalapril as his symptoms had resolved with discontinuation of this medication; however, we remained concerned that an underlying pulmonary process could also be present.The patient denied chest pain, orthopnea, paroxysmal nocturnal dyspnea, fever, weight loss, or night sweats. His testicular NHL was treated 16 years prior with orchiectomy and four cycles of CHOP without evidence of recurrence. In addition to losartan, the patient also took a daily aspirin. He was a retired carpenter who had a 19-pack year smoking history and had quit 37 years before presentation. He occasionally drank alcohol. He denied illicit drug use. On examination, he was not in distress. His weight was 80.2 kg. His temperature was 36.48C, pulse 130 and regular, respiratory rate 18, blood pressure 78/46, and oxygen saturation was 97% while breathing ambient air. Physical examination was remarkable for a normal jugular venous pressure, tachycardia without extra heart sounds and generalized anasarca including 2+ pitting edema of his lower extremities. There was no lymphadenopathy, palpable masses, or cutaneous lesions. His neurologic examination was normal.The patient's review of systems was negative for symptoms of an acute coronary syndrome or heart failure, as well as for B-symptoms or a rapidly progesssive infectious process. On physical examination, his vital signs suggested intravascular volume depletion with tachycardia and hypotension; however, he appeared to have a component of fluid overload or third spacing of fluids with the marked degree of anasarca. His normal jugular venous pressure suggested normal right heart pressure making a cardiac cause of his anasarca less likely.The patient's white cell count was 12,300/mm 3 , with 93% neutrophils, 1% bands, and 6% monocytes. The hemoglobin level was 11.3 g/dl, with a mean corpuscular volume of 78.8 mm 3 . The platelet count was 554,000/ mm 3 . The serum sodium level was 126 mmo...

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Section: Commentarymentioning

confidence: 99%

A 69‐year‐old male presenting with hypotension and anasarca

et al. 2007

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“…According to the most recent WHO classification, IVLBCL attained the status of an independent disease entity [17] after initially being classified as a rare form of extranodal diffuse large B-cell lymphoma [18]. A series of 96 IVLBCL patients reported the following clinicopathologic features at diagnosis: anemia/thrombocytopenia (84%), hepatosplenomegaly (77%), B symptoms (76%), bone marrow involvement (75%), and hemophagocytosis (61%) [11]. Most patients have an intermediate-high or high score on the International Prognostic Index [11].…”

Section: Discussionmentioning

confidence: 99%

“…A series of 96 IVLBCL patients reported the following clinicopathologic features at diagnosis: anemia/thrombocytopenia (84%), hepatosplenomegaly (77%), B symptoms (76%), bone marrow involvement (75%), and hemophagocytosis (61%) [11]. Most patients have an intermediate-high or high score on the International Prognostic Index [11]. Treatment outcomes have significantly improved with the addition of rituximab to standard anthracycline based chemotherapy.…”

Section: Discussionmentioning

confidence: 99%

“…Addition of rituximab to conventional chemotherapy improved complete remission (90% vs. 50%), event-free survival at 3 years (89% vs. 35%), and overall survival at three years (89% vs. 38%). The CNS is not infrequently involved (from 27% [11] to 39% [10]), either during the initial presentation or in relapse. Some groups have treated patients with highdose chemotherapy followed by autologous stem-cell transplantation either in first remission [20] or in the relapsed setting [21], which may lead to long-term remission [22], although widespread use of this approach is often limited by patient's performance status.…”

Section: Discussionmentioning

confidence: 99%

“…The next step in management should include staging with a PET/CT scan. Since the central nervous system is involved in 27-39% IVLBCL cases at diagnosis, this patient should also have CSF analysis as a part of the staging workup [10,11]. Involvement of the CNS will significantly alter therapy as well as prognosis.…”

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