Intravascular lymphomatosis: a clinicopathologic study of 10 cases and assessment of response to chemotherapy.

DiGiuseppe, Joseph A.; Nelson, William G.; Seifter, Eli; Boitnott, John K.; Mann, Risa B.

doi:10.1200/jco.1994.12.12.2573

Cited by 266 publications

(207 citation statements)

References 34 publications

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“…Indeed, in initial descriptions of intravascular LBCL, marrow involvement was described as an infrequent occurrence. 19,28 However, subsequent studies indicate that this disease involves the marrow with greater frequency than originally suggested. 20,25 In our patients with diffuse splenic involvement by LBCL, there was a predominant, but not exclusive intrasinusoidal pattern of marrow infiltration.…”

Section: Discussionmentioning

confidence: 94%

“…18 This unusual distribution of lymphoma cells leads to protean clinical features secondary to small vessel occlusion including nephrotic syndrome, mental status changes, and shortness of breath. 19,20 Hematologic abnormalities, including autoimmune hemolytic anemia, have also been associated with this diagnosis. 4 Given the clinical features in our patients, including the presence of unexplained cytopenias and dyspnea, and the pattern of BM and splenic involvement a diagnosis of intravascular LBCL was considered.…”

Section: Discussionmentioning

confidence: 99%

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Diffuse large B-cell lymphoma with distinctive patterns of splenic and bone marrow involvement: clinicopathologic features of two cases

2005

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Two unusual cases of large B-cell lymphoma with predominant splenic and bone marrow (BM) involvement and similar clinical and histopathologic features are described. Both patients presented with nonspecific constitutional symptoms, unexplained cytopenias, and splenomegaly. Splenectomy revealed diffuse red pulp involvement by large B-cell lymphoma. The perisplenic lymph nodes were also involved diffusely with effacement of normal nodal architecture, excluding a diagnosis of intravascular large B-cell lymphoma. BM biopsies revealed striking erythroid hyperplasia without overt morphologic evidence of involvement by lymphoma. Immunoperoxidase staining of the marrow biopsies with antibodies to CD20 and erythroidassociated antigens revealed involvement by large B-cell lymphoma morphologically resembling the early pronormoblasts. In both cases there was prominent, but not exclusive, intravascular/intrasinusoidal lymphomatous marrow infiltration. These cases represent an unusual variant of large B-cell lymphoma with distinctive patterns of splenic and BM involvement. Furthermore, they underscore the difficulties in identifying intrasinusoidal marrow infiltration by lymphoma in H&E-stained biopsy slides and demonstrate that this pattern of marrow infiltration may be seen in cases of large B-cell lymphoma distinct from the intravascular variant. Keywords: diffuse large B-cell lymphoma; intravascular lymphoma; spleen; bone marrow; erythroid hyperplasia; non-Hodgkin lymphoma Diffuse large B-cell lymphoma (LBCL) can involve nodal or extranodal sites; however, initial presentation in the spleen occurs infrequently. 1 Typically when diffuse LBCL involves the spleen it forms discrete tumoral masses within the splenic parenchyma. 2 A recent report detailed other less common patterns of splenic involvement by LBCL including diffuse infiltration of the splenic red pulp. 3 The clinicopathologic features of diffuse LBCL with these unusual patterns of splenic involvement have yet to be fully elucidated. Described herein are the distinctive clinical and histopathologic features of two cases of splenic LBCL with diffuse red pulp infiltration and concurrent involvement of lymph nodes and bone marrow (BM). These cases had strikingly similar clinical presentations chiefly characterized by dyspnea, unexplained cytopenias, and splenomegaly. In both instances, splenectomy performed to alleviate the cytopenias revealed diffuse, homogeneous involvement of the splenic red pulp by diffuse LBCL. Although the pattern of splenic red pulp involvement and the clinical features raised the possibility of intravascular LBCL, in both cases there was parenchymal involvement of splenic hilar lymph nodes, excluding this diagnosis as defined by the most recent WHO criteria. 4 Furthermore, both cases had prominent BM involvement by lymphoma, which was difficult to detect by routine morphologic evaluation of hematoxylin and eosin (H&E)-stained biopsy slides. This difficulty was due both to the presence of prominent erythroid hyperplasia as well as a predominan...

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Section: Discussionmentioning

confidence: 94%

Section: Discussionmentioning

confidence: 99%

Diffuse large B-cell lymphoma with distinctive patterns of splenic and bone marrow involvement: clinicopathologic features of two cases

2005

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“…20 Prolongation of survival has been reported in patients with intravascular large B-cell lymphoma who are treated with chemotherapy. 21 In a review of 35 patients who received chemotherapy, 54% had a complete remission. 21 Median survival for those who did not respond to chemotherapy, or only partially responded, was 10.5 months.…”

Section: Discussionmentioning

confidence: 99%

“…21 In a review of 35 patients who received chemotherapy, 54% had a complete remission. 21 Median survival for those who did not respond to chemotherapy, or only partially responded, was 10.5 months. 21 Although the number of cases in our study is too small to develop broad conclusions about the clinical behavior of intravascular large B-cell lymphoma involving cutaneous hemangiomas, the relatively favorable outcome in these two cases may have resulted from cutaneous signs and symptoms leading to relatively early disease identification and treatment.…”

Section: Discussionmentioning

confidence: 99%

Intravascular large B-cell lymphoma involving hemangiomas: an unusual presentation of a rare neoplasm

et al. 2005

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We report the clinicopathological features of two cases of intravascular large B-cell lymphoma involving cutaneous hemangiomas. The cases were identified from the consultation files of two of the authors. Both patients were women, 64 and 55 years of age, who presented with long-standing cutaneous hemangiomas of the posterior scalp and left shoulder, respectively. The lesions were brought to medical attention by an increase in size and change in color. Biopsies and immunohistochemical evaluation of the hemangiomas revealed extensive involvement by intravascular large B-cell lymphoma. The neoplastic cells were diffusely positive for CD20 in both cases and negative for CD3, pan-cytokeratin (AE1/AE3), epithelial membrane antigen, S-100, Factor VIII-related antigen, CD34 and CD31. Disease was limited to the hemangiomas in both patients. Treatment consisted of chemotherapy (both patients) and adjuvant radiation therapy (one patient). One patient had a recurrence of disease 33 months after initial diagnosis, leading to an autologous stem cell transplant. The other patient is without evidence of disease 27 months after initial diagnosis. Although this is a rare neoplasm, it is important to consider intravascular large B-cell lymphoma in the differential diagnosis of vascular lesions containing intravascular neoplastic cells.

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“…The most frequent sites of involvement are the nervous system and the skin, although many other organs can be affected [1]. Although localization in the lung is not rare in this disease [2][3][4][5], pulmonary arterial hypertension (PAH) associated with IVL has been only rarely described [6,7]. We report herein one case of IVL revealed by a subacute and severe precapillary PAH in the presence of long-lasting fever.…”

Section: Introductionmentioning

confidence: 95%

Severe pulmonary arterial hypertension as initial manifestation of intravascular lymphoma: Case report

et al. 2005

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Intravascular lymphoma (IVL) is a rare and usually fatal disease that belongs to the class of high-grade malignant lymphomas and which is characterized by proliferation of neoplastic lymphoid cells exclusively within the lumina of small blood vessels. Its polymorphic and nonspecific clinical manifestations make antemortem diagnosis very difficult. We report herein a case of IVL revealed by fatal, precapillary, pulmonary arterial hypertension and associated with long-lasting fever. Extensive investigation of the usual causes of pulmonary arterial hypertension was negative. The diagnosis of intravascular lymphoma was made on postmortem analysis, revealing diffuse and sometimes complete obliterations of the lumina of small blood vessels by large B-cell lymphoma, including pulmonary capillaries. Thus, we propose that IVL must be added to the spectrum of etiologies of subacute pulmonary arterial hypertension, notably in the context of associated fever, both entities requiring emergency diagnosis. For this purpose, blood collected via pulmonary capillary-wedge aspiration for cytologic examination may be associated with right-sided heart catheterization when this latter procedure is suitable. Am.

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Intravascular lymphomatosis: a clinicopathologic study of 10 cases and assessment of response to chemotherapy.

Abstract: These findings suggest that IVL represents a high-grade non-Hodgkin's lymphoma (NHL) with a propensity for systemic dissemination, and that CR and long-term survival may result in patients treated with aggressive combination chemotherapy.

Cited by 266 publications

References 34 publications

Diffuse large B-cell lymphoma with distinctive patterns of splenic and bone marrow involvement: clinicopathologic features of two cases

Diffuse large B-cell lymphoma with distinctive patterns of splenic and bone marrow involvement: clinicopathologic features of two cases

Intravascular large B-cell lymphoma involving hemangiomas: an unusual presentation of a rare neoplasm

Severe pulmonary arterial hypertension as initial manifestation of intravascular lymphoma: Case report

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