Monocytoid B cell lymphoma associated with antibodies to myelin-associated glycoprotein and sulphated glucuronyl paragloboside.

Monocytoid B cell lymphoma (MBCL) is an immunologically and morphologically well-defined low-grade lymphoma with a predilection for lymph nodes of the parotid region. We describe an association of MBCL with anti-myelin-associated glycoprotein (MAG) polyneuropathy in a 53-year-old male. The diagnosis of stage IV MBCL with nodular bone marrow infiltration, Sjögren's syndrome and sensorimotor polyneuropathy was made in October 1996. Serum immunoelectrophoresis demonstrated IgMkappa paraprotein. This was then cross-reacted with epitopes of MAG and sulphated glucuronyl paragloboside (SGPG) on myelin sheaths, and detected by thin layer chromatography and Western blot. Direct immunofluorescence of a sural nerve biopsy showed loss of myelin fibres, segmental demyelinization and IgM deposits on the myelin sheaths. The cerebrospinal fluid was normal. After six cycles of chemotherapy (ChlVPP protocol), all the patient's haematological parameters normalized accompanied by an improvement in neurological signs. The improvement of the polyneuropathy after chemotherapy indicates that the autoimmune anti-MAG and anti-SGPG antibodies resulted from the neoplastic lymphoid proliferation.

Characterization of CD13 and CD33 surface antigen-negative acute myeloid leukemia.

From a cohort of 220 adults with newly diagnosed acute myeloid leukemia (AML), 8 (3.6%) exhibited a rare variant of aberrant membrane phenotype. It was characterized with typical myeloid morphologic and cytochemical patterns and absence of myeloid associated antigens (CD13, CD33, CD14, glycophorin A, CD61). According to the French-American-British criteria, disease in 5 patients was classified as M1 and in 3 patients as M2. CD34, CD38, HLA-DR, and CD45 were strongly expressed in 4 of 5, 3 of 3, 8 of 8, and 3 of 3 analyzed cases, respectively. CD7 antigen was strongly expressed in 4 of 6 patients. Except for predominance of male sex and high frequency of CD7 antigen expression, no other remarkable clinical or biologic characteristics were noted. Detected variant of AML with the unusual membrane phenotype (CD34+, HLA-DR-positive, CD38+, CD45+, CD7+) might represent an example of extreme asynchrony in sequences of morphologic and immunologic maturation or abnormal epitope expression on leukemic cell membrane molecules CD13 and CD33. Although the clinical significance of this AML variant is unclear, the existence of such cases demonstrates the continued need for simultaneous cytochemical and immunologic studies in the evaluation of acute leukemias.

Idiopathic thrombocytopenia associated with Wilson's disease.

We present the case of a 53 year-old patient with idiopathic thrombocytopenia associated with Wilson's disease. Idiopathic thrombocytopenia was diagnosed in August of 1994, and as the response to corticosteroid therapy was poor, the patient underwent a splenectomy in October of the same year. A liver biopsy, which was performed during the operation, showed Wilson's disease in the form of mild, chronic, active hepatitis. The serum ceruloplasmin was low, and the Kayser Fleischer's ring was positive. MRI of the brain showed cortical reductive changes with areas of copper accumulation in the white brain matter. An unusual presentation of Wilson's disease associated with idiopathic thrombocytopenia has not been published as of yet. The diagnosis of Wilson's disease was made at an advanced, adult age, which may implicate a heterozygous genetic configuration.

Angioimmunoblastic lymphadenopathy (AILD) with benign course.

A group of rare systematic lymphoproliferative disorders have been described under the heading of angioimmunoblastic lymphadenopathy with dysproteinaemia (AILD), from purely reactive to bona fide malignancies. Some patients exhibit a benign form of disease, but most exhibit an aggressive form with high mortality rate. We present two elderly patients with prominent constitutional symptoms, generalized lymphadenopathy, hepatosplenomegaly, diffuse maculopapular rash, polyclonal hypergammaglobulinaemia and immunohaemolytic anaemia. Lymph node biopsies showed features consistent with the diagnosis of AILD. The patients were treated with steroid and they are in complete remission 3.5 and 2.5 years, respectively after the diagnosis has been established.

[Non-Hodgkin's lymphomas of the skin]. / Nehockinski limfomi s lokalizacijom u kozu.

The specific cutaneous infiltrations were found in 20% of non-Hodgkin's lymphomas in the course of the disease, and they were its primary manifestation in 7.5% of cases. B cell and T cell lymphomas were equally represented in the primary manifestation, but B cell lymphomas prevailed in the secondary. Eight cases of cutaneous malignat lymphomas are presented: three of them are primary and five are secondary cutaneous lymphomas. Histologocally, there are two cases of low grade, four cases of intermediate grade, and two cases of high grade of malignance according to Working Formulation Criteria. The patients were treated with various therapies. The results suggest the conclusion that follicular cutaneous lymphomas and lymphomas of any other localization have better prognosis.