Successful treatment of relapse of an intravascular B-cell lymphoma with rituximab-CHOP polychemotherapy

Autor: Zsuzsánna Károlyi, I. Bene, Istvan Takacs, Nóra Eros, G. Radványi, András Matolcsy, L. Tordai, F. Bozso
Rok vydání: 2004
Předmět:
Zdroj: Annals of Hematology. 83:608-610
ISSN: 1432-0584
0939-5555
DOI: 10.1007/s00277-004-0897-0
Popis: In intravascular (angiotropic) lymphoma, malignant, mainly B-cell proliferation presumably can be found within the small vessels of the skin or the central nervous system [5]. Obliterations of capillaries, venules, and small arteries are responsible for the clinical signs. Erythematous papules, teleangiectasias, and paresthesias as well as other bizarre neurological signs can be seen [8]. During progression secondary organ involvement could also appear. Most of the cases are diagnosed by postmortem examination. Our patient, a 79-year-old female, had suffered from hypertension since 1994. She was treated in many outpatient facilities because of hemihypesthesia, short aphasic episodes, lower limb paresthesias, and painful erythematous papules of the skin. She received topical corticosteroid treatment for her skin lesions. She was admitted to the Dermatological Department of our hospital in 1998 because of generalized teleangiectasias, edema, and “orange-like” skin phenomenon. There was no palpable hepatomegaly, splenomegaly, or lymphadenomegaly. The computed tomography (CT) scan of the whole body was negative. Laboratory tests showed highly elevated lactate dehydrogenase levels (5804 U/l, normal range: 230–460 U/l), elevated serum β2-microglobulin (5.17 mg/l, normal range: 0.9–2.5 mg/l) levels, and accelerated sedimentation rate (32 mm in 1 h). Blood test showed mild anemia (red blood cell count: 3.69×10/ l, hemoglobin level: 10.5 g/dl, packed cell volume: 0.32). The platelet count was 126×10/l, and the total protein was 53 g/l with albumin content of 33 g/l. The examination of peripheral blood smear and bone marrow aspiration revealed the existence of a malignancy-associated secondary myelodysplasia. We did not find any cytogenetic aberrations. Serology for Borrelia burgdorferi was negative. The histopathological analysis of the skin biopsy specimen from the abdominal skin lesion revealed perivascular lymphoid infiltration. Numerous dilated capillaries were seen in the dermis containing many huge atypical centroblast-like lymphoid cells with nuclei containing one or two nucleoli. Fibrin deposits were seen between the intravascular cell groups. According to the immunohistochemical analysis, intravascular cells were identified as CD45, CD20, CD79a, and HLADR B-cells with high proliferation (MIB-1) activity (Table 1). Right after the proper diagnosis, the patient received systemic treatment with psoralen ultraviolet A-rays (PUVA) and then chlorambucil 2×5 mg weekly. Her edema and teleangiectasias disappeared, laboratory parameters normalized, neurological symptoms ended, and she achieved complete remission. The patient relapsed with almost the same symptoms described above in 2000. After administration of three cycles of CHOP chemotherapy, complete remission was achieved again. Maintenance therapy with interferon-alpha (3×4.5 MIU/week) was applied successfully. The patient was admitted to our department because of a 1-week history of left leg swelling in May of 2003. At admission she had aphasia, hemihypesthesia of the left side, and multiple teleangiectasias on the chest, abdomen, and the lower extremity (Fig. 1). Color-coded Doppler ultrasonography showed a deep vein thrombosis in the iliofemoropopliteal region of the left leg combined with I. Takacs (*) . I. Bene . F. Bozso . G. Radvanyi 2nd Department of Internal Medicine and Hematology, Semmelweis Hospital, Csabai kapu 9-11, 3501 Miskolc, Hungary e-mail: itakacs.2bel@semmelweis-miskolc.hu Tel.: +36-46-555667 Fax: +36-46-555667
Databáze: OpenAIRE
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