Gilles Lugassy

The heterogeneity of Castleman disease: report of five cases and review of the literature.

Castleman disease (CD; angiofollicular lymphoid hyperplasia) is a heterogeneous group of lymphoproliferative disorders of uncertain cause. Three histologic variants (hyaline vascular, plasma cell, and mixed) and two clinical types (localized and multicentric) of CD have been described. We report 5 cases of CD treated in our institute and review the literature about the management of this relatively rare disorder. Localized and multicentric CD may be different clinical disorders with overlapping histologic features. Localized disease generally presented with a single enlarged lymph node or widening of the mediastinum, whereas multicentric disease is a systemic lymphoproliferative disorder characterized by lymphadenopathy, hepatosplenomegaly, constitutional symptoms, anemia, hypoalbuminemia, and hypergammaglobulinemia. Unlike the localized type, for which surgical excision is curative regardless of the histologic type, multicentric disease often necessitates aggressive systemic therapy and portends a poorer outcome.

Clinical Implications of Fluorescence In Situ Hybridization Analysis in 13 Chronic Myeloid Leukemia Cases: Ph-Negative and Variant Ph-Positive

Thirteen chronic myeloid leukemia (CML) patients, 10 with variant Philadelphia (Ph) translocations and 3 Ph negative cases, were analyzed by fluorescence in situ hybridization (FISH) with the use of BCR and ABL cosmid probes and a chromosome 22 painting probe. In the variant Ph translocations, the BCR-ABL fusion gene was located on the Ph chromosome; in 1 CML Ph-negative patient, the BCR-ABL fusion gene was located on the Ph chromosome; and, in 2 patients, it was located on chromosome 9. The chromosome 22 painting probe was detected on the third-party chromosome of the variant translocation, and in none of the variant translocations was there any detectable signal on chromosome 9. In CML patients with clonal evolution of a simple Ph, a signal of the chromosome 22 painting probe was detected on the der(9) of the Ph translocation. It was concluded that the variant Ph translocations evolved simultaneously in a three-way rearrangement. The clinical parameters of the 13 patients were similar to those of a large group of CML patients with a simple Ph translocation. It is suggested that, to determine the prognosis of CML patients with a complex karyotype, FISH analysis with a chromosome 22 painting probe be performed.

Case Report: Severe Pyoderma Associated with Familial Mediterranean Fever—Favorable Response to Colchicine in Three Patients

Familial Mediterranean fever (FMF) is an autosomal recessive disorder that occurs most frequently among Sephardic Jews and Armenians. It is characterized by recurrent episodes of fever, peritonitis, pleuritis, and arthritis. Skin lesions are seen in some patients. Diagnosis of FMF usually is made on clinical grounds only, typically when recurrent attacks of abdominal pain, fever, and arthritis are observed in a patient with an appropriate ethnic background and family history. To date, there are no specific diagnostic laboratory tests for FMF. Three patients with severe recurrent Pyoderma are covered in this report. In all three cases, the cutaneous lesions were associated with clinical manifestations of FMF and responded to colchicine therapy favorably. The importance of such an association and its therapeutic consequences are emphasized.

Severe autoimmune hemolytic anemia with cold agglutinin and sclerodermic features — favorable response to danazol

SummaryA rare case of severe primary autoimmune hemolytic anemia with cold agglutinin and extensive cutaneous sclerodermic changes is reported. This association has not been previously documented in the literature. The anemia was refractory to steroids but responded to danazol treatment. Danazol may be an effective therapy in some cases of autoimmune hemolytic anemia with cold agglutinin.

Hypoglycemia-induced hemolysis in glucose-6-phosphate dehydrogenase deficiency.

We present a young man with Mediterranean type glucose-6-phosphate dehydrogenase (G6PD) deficiency and insulin-dependent diabetes mellitus whose brittle course was characterized by recurrent bouts of hypoglycemia and diabetic ketoacidosis (DKA). While neither of the episodes of DKA was complicated by hemolysis, hemolytic anemia consistently followed the recurrent attacks of hypoglycemia. Stringent control of the patients blood glucose levels in the upper limit and slightly above the normal range successfully prevented recurrence of hypoglycemia and recrudescence of hemolytic anemia. Hypoglycemia is proposed as capable of inducing hemolysis in G6PD deficiency.

Non-Hodgkin's lymphoma presenting with amegakaryocytic thrombocytopenic purpura

Abstract A 91-year-old patient was diagnosed with amegakaryocytic thrombocytopenic purpura (AATP) as a presenting symptom for CD5-positive B cell non-Hodgkins lymphoma. Lymphoma is another condition that should be considered in the differential diagnosis of AATP.

Polycythemia as a result of ectopic erythropoietin production in benign cystic leiomyoma of uterus

A 33-year-old woman had a symptomatic uterine myoma for at least 1 year. Physical examination revealed facial flushing and a pelvic mass extending above the umbilicus. Spleen was not enlarged. Abdominal sonography, computerized tomography and magnetic resonance imaging demonstrated a pelvic mass measuring 24 /22 /11 cm. In order to preoperatively decrease the size of the myoma, triptorelin depot was administered for 3 months. Under this regimen, the maximal diameter of the myoma decreased to 20 cm. The hematocrite and hemoglobin levels were extremely elevated, 71% and 23 g/dl, respectively. Other blood cell lines were normal. Biochemical laboratory tests revealed hyperuricemia (6.4 mg/dl). Arterial blood gases showed normal oxygen tension and saturation level. Plasma erythropoietin level was 185 mU/ml (reference values: 3.3 16.6 mU/ ml). Echocardiography examination revealed completely normal cardiac structure and function. In order to prevent possible perioperative thrombotic complications, the patient underwent 10 phlebotomies, about 400 ml each. When the hematocrite level reached 47%, she was referred to surgical treatment. Discussion

Chronic Paraneoplastic Cutaneous Syndrome Preceding an Indolent Variant of Mantle Cell Lymphoma: Favorable Response to Rituximab

Mantle cell lymphoma (MCL) is an aggressive type of malignant lymphoma with short median survival despite conventional therapy. We describe the unusual case of a 66-year-old man with a chronic skin rash which preceded the occurrence of an indolent MCL by 2 years. Repeated skin biopsies did not show involvement by the lymphoma. Short therapy with rituximab resulted in the complete and lasting resolution of the cutaneous lesions.

Recurrent Thrombophlebitis Due to Protein C Deficiency after Splenectomy for Immune Thrombocytopenic Purpura

A young woman with recurrent thrombophlebitis following splenectomy for immune thrombocytopenic purpura and two cesarian sections was found to carry a protein C deficiency. This pathology should be considered in any patient with recurrent thromboembolism even when predisposing factors such as pregnancy and postoperative state may by themselves be responsible for such manifestations.

Chronic Lymphocytic Leukemia Associated with Systemic Lupus erythematosus

Dr. Gilles Lugassy, Institute of Hematology, Barzilai Medical Center, 78306 Ashkelon (Israel) Lishner et al. [1] have recently described, in this Journal, the case of a 48-year-old woman who developed chronic lymphocytic leukemia (CLL) 5 years after she was diagnosed as suffering from systemic lupus erythematosus (SLE). The authors emphasize the rarity of such an association and review the few similar cases published in the literature, mainly before 1970. We wish to complete this review of the literature and briefly report two identical cases which were recently published [2]. The first patient was a 72-year female who developed SLE (Coombs-positive hemolytic anemia, arthritis, antinuclear factor, LE cells) 2 years after diagnosis of CLL. She later succumbed to an inoperable gastric carcinoma. The second patient was a 70-year-old man whose diagnosis of CLL was arrived at while he was hospitalized because of pleuritic pain and ar-thralgia. Antinuclear factor was strongly positive and LE cells were abundant in the peripheral blood. These two cases of coexistent CLL and SLE tend to confirm that this association is not coincidental as emphasized by Lishner et al. [1]. References Lishner M, Hawker G, Amato D: Chronic lymphocytic leukemia in a patient with systemic lupus erythematosus. Acta Haematol 1990;84:38–39. Michaeli J, Lugassy G, Raz I, Polliack A: Chronic lymphocytic leukemia, lymphoma and autoimmunity; in Polliack A, Ca-tovsky D (eds): Chronic Lymphocytic Leukemia. London, Harwood Academic Publishers, 1988.