Intravascular large b-cell lymphoma C83.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 10.11.2021

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Angioendotheliomatosis; Angioendotheliomatosis proliferans systematisata; Intravascular B-cell lymphoma; Intravascular B-cell lymphoma of the skin; Intravascular large B-cell lymphoma; Intravascular large B-cell lymphoma of the skin; Intravascular large cell cutaneous B-cell lymphoma; Intravascular Lymphoma; IVL; malignant angioendotheliomatosis

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Extremely rare B-cell lymphoma; usually systemic with proliferation of multicentric, intravascular tumor cell infiltrates leading to occlusion of small and medium-sized vessels of different organs. Systemic intravascular lymphomas affect the integument in about 40%. Isolated integumentary involvement of intravascular lymphomas is very rare. Characteristic for the skin involvement of all intravascular lymphomas is the histological pattern with strictly intravascular tumor cell volumes that do not infiltrate the surrounding tissue.

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There is a rare, purely cutaneous form of progression, which is listed as a separate entity in the current WHO classification under the name " intravascular large B-cell lymphoma".

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Intravascular B-cell lymphoma is an extremely rare disease that leads to occlusion of small and medium-sized vessels with proliferation of multicentre intravascular tumour cell infiltrates. The loss of lymphocyte function antigens prevents the extravasation of lymphoma cells. The diagnosis of intravascular B-cell lymphoma is made histologically and immunohistologically. The clinical symptoms are very heterogeneous. Treatment is performed according to the protocols of nodal B-cell lymphomas.

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Men and women fall ill equally often. Mostly occurring after the 60th LJ.

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Especially the lower leg and lower abdomen are affected.

Clinical features
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The clinical picture is limited to the skin, variegated and clinically not very suggestive. Mostly formation of solitary, asymptomatic (occasionally also painful), blurred, reticular or homogeneous, extensive erythema. However, disseminated, indurated, small or large plaques have also been described, as have subcutaneous, sometimes panniculitic nodules. Rarer are localized papular foci interspersed with petechiae or telangiectasias. The skin manifestations of the purely cutaneous or the systemic form of intravascular lymphoma do not differ essentially.

General: Fever, B-symptomatics, variable systemic involvement (especially lymph nodes, CNS with neurological deficits) may occur.

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No landmark lab parameters. If gamma globulins are elevated, monoclonal gammopathy should be detected.

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Intravascular pleomorphic B-cell lymphocytes with round or oval chromatin dense nuclei. Blood vessels in the dermis, sometimes also in the subcutis (see case report), are dilated and increasingly filled with highly pleomorphic, hyperchromatic cells. The cells may be embedded in fibrinous thrombi. Immunohistologically, these cells characterize as B lymphocytes(CD19, CD20, pos.; high proliferation rate). Evidence of a clonal lymphocyte population. They do not express antigens characteristic of endothelial cells. Note: Loss of lymphocyte function antigens prevents extravasation of lymphoma cells!

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Only possible in connection with clinic, histology and immunohistology. Dermatological main symptom: Rough indolent nodulation of cutaneous vessels with thrombophlebitis.

Differential diagnosis
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The therapy is similar to protocols for other nodal B-cell lymphomas. Due to the rarity of intravascular lymphoma there are no independent therapy protocols. In smaller case series, treatment success was achieved by CHOP chemotherapy, possibly in combination with rituximab analogous to the treatment of extranodular large B-cell lymphomas (see case report). Rituximab was also used as a single protocol.

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The very rare purely cutaneous variant of intravascular lymphoma ("lymphoma, cutaneous B-cell lymphoma, intravascular, large cell") is listed as an entity in the current WHO classification.

In this compilation, intravascular lymphoma is considered a clinically and prognostically heterogeneous group of lymphomas with a variable cutaneous pattern of involvement characterized by a uniform histologic pattern. Case reports exist of T-cell - or NK-cell-associated intravascularlymphoma (lymphoma, cutaneous T-cell lymphoma, intravascular).

Originally, intravascular lymphoma (IVL) was misinterpreted as a tumor of endothelial cells. This resulted in the older term "malignant angioendothelioma" or "angioendotheliomatosis proliferans systematisata" (see below Angioendothelioma). The primary systemic angioendotheliomatosis proliferans systematisata was contrasted in the older literature with the benign or reactive angioendotheliomatosis " angioendotheliomatosis, reactive (benign)". This disease corresponds with that of the purely cutaneous form of intravascular lymphoma.

Case report(s)
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In a 64-year-old female patient, indolent skin-coloured swellings suddenly appeared in the area of both legs; evidence of 2 painless, approximately 3 cm large lumps on the right thigh. Clear B-symptoms with weight loss of about 15 kg in the last 6 months. Diagnostics: Laboratory: Completely inconspicuous. Examination of bone marrow, MRI skull, PET-CT, chest and upper abdomen sonography, duplex sonography: Insufficiency of the venae saphenae magnae, grade III n. Hach; mainly for thrombosis of the lateral branches of the vena saphena magnae rechs. Histology (Nodules: Intravascular pleomorphic B-cell lymphocytes with roundish chromatin dense nuclei). In addition partial thrombotic occlusion of the venules and microthrombi in the capillaries. Immunohistologically positive for CD20 and CD79. Ki 67: Intravascular lymphocytes with extremely high proliferation rate (about 70 to 80%.) Detection of a B-cell clone. Therapy and course: Initiation of a therapy according to the R-CHOP protocol (rituximab, cyclophosphamide, hydroxy-doxorubicin, vincristine, prednisolone). In addition, the patient received enoxaparin 2x80 mg/day; accompanying consistent compression therapy. A total of 6 cycles; RE staging after 6 months: no evidence of lymphoma; rapid improvement of clinical symptoms.

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  1. Asagoe K et al (2003) Intravascular lymphomatosis of the skin as a manifestation of recurrent B-cell lymphoma. J Am Acad Dermatol 48: S1-4
  2. Crane GM et al (2014) Primary effusion lymphoma presenting as a cutaneous intravascular lymphoma. J Cutan Pathol 41: 928-935.
  3. Han K et al (2003) Regression of cutaneous intravascular lymphoma with rituximab. Cutis 72: 137-140
  4. Kamath NV et al (2001) Primary cutaneous large B-cell lymphoma of the leg relapsing as cutaneous intravascular large B-cell lymphoma. Arch Dermatol 137: 1657-1658
  5. McDonough SP et al (2002) Clinicopathological and immunophenotypical features of canine intravascular lymphoma (malignant angioendotheliomatosis). J Comp Pathol 126: 277-288
  6. Samols MA et al (2014) Intralymphatic cutaneous anaplastic large cell lymphoma/lymphomatoid papulosis: expanding the spectrum of CD30-positive lymphoproliferative disorders. On J Surg Pathol 38:1203-1211.
  7. Satzger I et al (2009) Intravascular B-cell lymphomas. Dermatologist 60: 131-136
  8. Willemze R et al (2005) WHO-EORTC classification for cutaneous lymphomas. Blood 105: 3768-3785


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Last updated on: 10.11.2021