Intravascular Large B-Cell Lymphoma—A Diagnostic Dilemma

Ramkumar, B.; Hutchison, Robert; Khadim, Haider; Gajra, Ajeet

doi:10.1016/j.clml.2013.07.001

Cited by 10 publications

(4 citation statements)

References 13 publications

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“…[11] The most common symptoms are B symptoms such as fever, night sweats, and weight loss, as well as progressive neurologic symptoms, skin lesions, general fatigue, gastrointestinal discomfort, and edema. [4,5,12,13] There are 2 major patterns of clinical presentation.…”

Section: Discussionmentioning

confidence: 99%

Intravascular large b-cell lymphoma diagnosed via transjugular liver biopsy in a patient with liver dysfunction and thrombocytopenia

Kim¹,

Park

Yhim

2017

Medicine

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Rationale:Intravascular large B-cell lymphoma (IVLBCL) is an extremely rare subtype of large B-cell lymphoma characterized by the presence of lymphoma cells within the lumen of small blood vessels. IVLBCL presents with nonspecific symptoms such as fever, weight loss, and bleeding. Because of its rarity and unremarkable clinical presentation, a timely diagnosis is very challenging.Patient concerns:A 71-year-old Korean man complained of fever, but apart from pretibial pitting edema and mild thrombocytopenia, the physical examination and laboratory test findings were unremarkable.Diagnoses:A bone marrow biopsy was also nonspecific. The fever persisted and his thrombocytopenia became more pronounced, prompting further laboratory tests that indicated infiltrative liver disease.Interventions:Because of coagulopathy, a liver biopsy was performed using a transjugular instead of apercutaneous approach.Outcomes:The procedure was performed without complications, and the pathologic examination findings were consistent with IVLBCL. Unfortunately, the patient died because of disease progression before treatment could be administered.Lessons:Given that an early diagnosis can affect the prognosis of IVLBCL, quickly and safely obtaining a biopsy specimen is very important. The case presented here shows that a liver biopsy obtained via a transjugular approach is safe and could be preferentially considered when there is a high risk for bleeding complications.

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Section: Discussionmentioning

confidence: 99%

Intravascular large b-cell lymphoma diagnosed via transjugular liver biopsy in a patient with liver dysfunction and thrombocytopenia

Kim¹,

Park

Yhim

2017

Medicine

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“…Prophylaxis with intrathecal chemotherapy has resulted in varied outcomes [ 24 – 27 ]. Two cases of IVL without CNS involvement at diagnosis achieved complete remission without CNS relapse for 20 months in the patient that received four doses of intrathecal MTX and 21 months in the patient that received intrathecal MTX, cytarabine, and dexamethasone [ 25 , 26 ]. However, even though a patient with IVL of the penis, prostate, and bones without CNS involvement at diagnosis achieved complete remission after six cycles of systemic treatment with R-CHOP and four doses of intrathecal MTX for CNS prophylaxis, the patient ultimately experienced CNS relapse after 6 months [ 24 ].…”

Section: Therapeutic Interventionsmentioning

confidence: 99%

Intravascular Lymphoma in the CNS: Options for Treatment

Nizamutdinov

Patel

Huang

et al. 2017

Curr Treat Options Neurol

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Purpose of review The purpose of this review was to discuss therapeutic manipulations and effective current interventions available to treat intravascular lymphoma in the central nervous system. Recent findings Patients experienced resolution and remission of disease for 14 months and up to 2 years after eight cycles of R-CHOP and four courses of intrathecal therapy with MTX, cytarabine, and prednisolone. Intravascular use of unfractionated heparin during therapy may contribute to better outcome. Summary Series of therapeutic avenues were analyzed and compared. The effective current treatment of intravascular lymphoma in the CNS is considered to be a combinational intrathecal methotrexate-based chemotherapy with rituximab. Since intrathecal administration bypasses the blood–brain barrier, lower doses can be given, which thereby minimizes systemic toxicity. Practical use of intrathecal chemotherapy is also justified for prophylaxis in intravascular lymphoma-diagnosed patients without CNS involvement.

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“…2 It was first described by Lorenz Pfleger and Jasmin Tappeiner in 1959 as vascular endothelial neoplasm, namely, “angioendotheliomatosis proliferans systemisata.” 3 Its rarity, unique presentation in extranodal organs, and unsuspected clinical presentation pose a challenge to timely diagnosis and management. 4 This is compounded by the fact that there is no definite organ to biopsy. Therefore, most patients were diagnosed at autopsy in the past.…”

Section: Introductionmentioning

confidence: 99%

The Rare Face of an Infrequent Lymphoma Diagnosed by an Exquisite Method: Case Report with Review of Literature

Lali

Dey

Bhave

et al. 2021

Indian J Med Paediatr Oncol

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Large B-cell lymphomas include both the commonly encountered, as well as not so frequent entities. We report a rare case of hepatic intravascular large B-cell lymphoma (IVLBCL). These cases usually pose a major challenge to timely diagnosis due to the limited clinical exposure and nonspecific symptoms; however, timely diagnosis is possible with awareness of this entity and close coordination between radiology and pathology. The patient presented with pain abdomen, fever, edema, hepatomegaly but no lymphadenopathy or cutaneous lesions were identified. Blood investigations revealed pancytopenia, high lactate dehydrogenase (LDH) levels and bone marrow investigations were not helpful. Left-sided portal hypertension, coagulopathy, and hypoalbuminemia raised a clinical suspicion of chronic liver disease with high LDH levels and liver biopsy was challenging in view of very low platelet counts. The uniquely explored transjugular biopsy helped in obtaining liver tissue which showed sinusoidal large atypical lymphoid cells positive for CD20 rendering a diagnosis of IVLBCL. After administration of the R-miniCHOP chemotherapy, there was evident clinical and radiological response. This case highlights the importance of considering transjugular liver biopsy in scenarios where the clinical presentation is unusual. At the same time, a meticulous histological examination is needed to diagnose a rare yet potentially treatable large B cell lymphoma.

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Intravascular Large B-Cell Lymphoma—A Diagnostic Dilemma

Cited by 10 publications

References 13 publications

Intravascular large b-cell lymphoma diagnosed via transjugular liver biopsy in a patient with liver dysfunction and thrombocytopenia

Intravascular large b-cell lymphoma diagnosed via transjugular liver biopsy in a patient with liver dysfunction and thrombocytopenia

Intravascular Lymphoma in the CNS: Options for Treatment

The Rare Face of an Infrequent Lymphoma Diagnosed by an Exquisite Method: Case Report with Review of Literature

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