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Biomedical subjects

Y Matzner

Publications and source records attributed to Y Matzner.

At least 109 records · Page 6Linked to original sources

Acute lymphoblastic leukemia. Association with vasopressin-responsive diabetes insipidus.

A rare case of acute lymphoblastic leukemia presenting with vasopressin-responsive diabetes insipidus (DI) is reported. The patient presented with polydypsia and polyuria of 9 L/day. Findings from special investigations of the CNS, including brain scan, computerized tomographic scan, EEG, and lumbar puncture were within normal limits. The patient's condition improved substantially after receiving vasopressin injections and later chlorpropamide. The incidence and underlying mechanism of this rare complication of acute leukemia are reviewed, and the response of DI to chlorpropamide is discussed briefly. In this patient it is presumed that the DI was caused by leukemic infiltration of the supraopticohypophyseal tract, posterior pituitary, or hypothalamus.

Adult↗

Serum ferritin in hematologic malignancies.

Serum ferritin was measured in a variety of hematologic malignancies at presentation, in remission following therapy, and in relapse. Ferritin was strikingly increased in all acute leukemias at presentation and in relapse, in the blastic crisis of CML, and in smouldering leukemia. Remission in both ALL and ANLL was associated with a reduction of serum ferritin, and this normalization was a function of remission duration. In the malignant lymphomas serum ferritin was related to tumor histology. Highest levels were found in Hodgkin disease and histiocytic lymphoma, normal levels in lymphocytic lymphoma, and intermediate levels in mixed histiocytic-lymphocytic lymphoma. In all cases, remission was associated with normalization of serum ferritin. These correlations suggest that serum ferritin measurements may be of clinical usefulness in the initial evaluation and in the assessment of response to therapy in patients with acute leukemia and malignant lymphoma.

Acute Disease↗

Lymphoblastic leukemic transformation (lymphoblastic crisis) in myelofibrosis and myeloid metaplasia.

Acute lymphoblastic leukemia (ALL) developing in myelofibrosis (MF) and myeloid metaplasia (MM) is reported in two patients. In both cases, the clinical course of the "blastic crisis" was rapidly progressive with little response to chemotherapy. The circulating cells were readily identified as lymphoblasts on the basis of cytology, cytochemistry, immunologic studies, and ultrastructure. In one of the cases, 40% of cells had T-cell markers and all cells contained paranuclear acid phosphatase. In the second case, cells had a "Burkitt-like appearance, contained multiple cytoplasmic vacuoles positive for oil red O, and one-third of them had B-cell markers. The development of lymphoblastic crisis in MF and MM occurs rarely, is analogous to blastic transformation in chronic granulocytic leukemia, and supports the hypothesis that myeloproliferative disorders originate from pluripotent hematopoietic stem cells.

Cell Transformation, Neoplastic↗

Bone marrow necrosis as the only manifestation of disseminated carcinomatosis.

An unusual case with thrombocytopenia and bone marrow necrosis as the only manifestation of disseminated carcinomatosis is reported. The diagnosis was first established by repeated bone marrow biopsy which revealed marked necrosis without evidence of malignancy. The patient's general condition deteriorated rapidly despite therapy with steroids. Autopsy revealed disseminated metastatic adenocarcinoma, but the primary site of the tumor was not identified. It is suggested that malignancy should always be excluded in patients who have bone marrow necrosis without an obvious cause.

Adenocarcinoma↗

Fatal Shigella dysentery complicated by toxic megacolon and bone marrow aplasia in a patient with chronic granulocytic leukemia in remission.

A patient with Philadelphia positive chronic granulocytic leukemia in clinical remission is described, who developed Shigella dysentery complicated by fatal toxic megacolon, pancytopenia and bone marrow aplasia. The difficulties of differential diagnosis between active ulcerative colitis and Shigella dysentery and problems relating to the management of these two disorders are discussed. Leukocyte function in chronic granulocytic leukemia and its role in infection in these patients is also briefly reviewed. The rare association of bone marrow aplasia and Shigellosis is stressed.

Anemia, Aplastic↗

Systemic leishmaniasis mimicking malignant histiocytosis.

A 22-year-old man with fever, hepato-splenomegaly and severe pancytopenia is described in whom the histologic features of the spleen, liver and lymph nodes were consistent with malignant histiocytosis. Characteristic Leishman-Donovan bodies were demonstrated on a bone marrow aspirate, and the diagnosis of visceral leishmaniasis was confirmed by culturing the flagellated forms of the pathogen, and by an excellent response to sodium stibogluconate. In view of the similarity in clinical and histologic appearance, visceral leishmaniasis must be considered in the differential diagnosis of malignant histiocytosis in geographic areas where leishmaniasis is still endemic.

Adult↗

Suppressive effect of ferritin on in vitro lymphocyte function.

This study describes the effect of ferritin on lymphocyte function in vitro. Peripheral blood lymphocytes isolated from normal donors were incubated with purified human splenic ferritin, and the mitogenic effect of phytohaemagglutinin (PHA), concanavalin A (Con A), pokeweed mitogen (PWM) and mixed lymphocyte reaction (MLR) were assessed by the uptake of 3H-thymidine (3H-TdR). Ferritin (0.25--5.0 micrograms/ml culture) caused a marked suppression of PHA nad Con A blastogenesis but had no suppressive effect on PWM-induced transformation. Maximal suppression was obtained at a ferritin concentration of 1 microgram/ml and this was not enhanced by increasing ferritin concentrations. Ferritin also reduced the Con A capping phenomenon in normal lymphocytes from 22% to 6%, suppressed the MLR reaction but had no effect on the ability of normal lymphocytes to form E, EA and EAC rosettes or on in vitro lymphocyte cytoxicity against the K-562 cell line. Visual proof of the suppressive effect of ferritin on mitogen induced blastogenesis was provided by scanning electron microscopy, and direct evidence for the ability of lymphocytes to bind ferritin was obtained from studies with radioiodine labelled ferritin. The above findings indicate that ferritin suppresses certain parameters of T-lymphocyte function in vitro. The relation of the present findings to recognized abnormalities of T-cell function encountered in certain neoplastic disorders associated with high serum ferritin levels is at present unknown.

Cytotoxicity, Immunologic↗

Prevalence and causes of anemia in elderly hospitalized patients.

The prevalence and causes of anemia have been studied in 104 patients over 60 years of age admitted to a general medical ward in Jerusalem. In males and females, mean hemoglobin levels were about 1 g less than in the corresponding groups of healthy younger controls. A primary nutritional anemia could not be implicated in any of the 15 patients with hemoglobins below 11 g/dl. The most important causes of anemia were chronic renal failure, metastatic carcinoma, gastrointestinal bleeding, and infection. Conversely, in diseases with no adverse effect on erythropoiesis such as chronic ischemic heart disease, hypertension and diabetes, hemoglobin levels were equal to those of the younger controls. These findings indicate that although diminished serum iron and RBC folate levels may occasionally be found in elderly subjects, nutritional deficiency is seldom responsible for anemia in this age group in Israel- and anemia when present is often the manifestation of a chronic underlying disease.

Adult↗

Myelomatosis after phenytoin therapy: a chance association?

The occurrence of a monoclonal gammopathy followed by IgG-lambda multiple myeloma is described in a patient receiving diphenylhydantoin therapy for 20 years for epilepsy. Despite the known association between the drug and the development of immunosuppression and lymphoma, we regard the association recorded in this case to be coincidence. It is suggested that periodic examination of the serum proteins in patients receiving the drug may be useful in detecting early aberrations of the immune system in these individuals.

Agammaglobulinemia↗

Prognostic factors in multiple myeloma: a retrospective study using conventional statistical methods and a computer program.

The prognostic significance of age, sex, ethnic origin and various laboratory data was studied retrospectively in 69 patients with multiple myeloma using conventional statistical tests and the multiple regression computerized analysis. The conventional statistical analysis confirmed that age, anemia, uremia, hypoalbuminemia, hyperglobulinemia, hyperuricemia and IgA lambda type myeloma were associated with a poor prognosis. The multiple regression analysis indicated that age and blood urea nitrogen levels were the only variables which significantly affect the survival of patients with multiple myeloma. A correlation was found between blood urea nitrogen levels and other laboratory data of apparent prognostic value. The differences between our results and those of other authors are discussed and it is suggested that they may, in part, be due to the fact that the interplay between the various prognostic variables was not exposed in other studies.

Age Factors↗

Lymphoproliferative disorders in four patients receiving chronic diphenylhydantoin therapy: etiologic correlation or chance association?

The occurrence of angioimmunoblastic lymphadenopathy, Hodgkin's disease or IgG-lambda multiple myeloma is described in four patients receiving chronic diphenylhydantoin (DILANTIN) therapy. Although the association between diphenylhydantoin therapy and the development of immunosuppression and lymphoma is well documented, the role of the drug in the etiology of these disorders is still controversial. It is suggested that periodic examination of patients receiving diphenylhydantoin for lymphadenopathy and repeated serum electrophoresis may be useful in detecting early aberrations of the immune system in these individuals.

Adult↗

Hodgkin's disease and subsequent chronic lymphocytic leukemia in a patient with breast carcinoma.

This report deals with an unusual case of a patient with four primary tumors. Hodgkin's disease developed in an elderly woman 21 years after she was treated for carcinoma of the breast by surgery and irradiation. Chronic lymphocytic leukemia and a liposarcoma of the soft tissues developed two years after the appearance of Hodgkin's lymphoma. The coexistence of four primary tumors in the same patient is rare; the simultaneous occurrence of Hodgkin's lymphoma and lymphocytic leukemia is in itself a rare association and is probably a chance finding. The development of these neoplasias may in some way relate to the radiotherapy given to the patient.

Breast Neoplasms↗

Monoclonal gammopathy and subsequent multiple myeloma in a patient on chronic diphenylhydantoin therapy.

The development of an IgG lambda-type monoclonal gammopathy and subsequent multiple myeloma in an epilepsy patient on diphenylhydantoin (DILANTIN) therapy for 20 years is reported. We regard the association recorded in this case to be a coincidence despite the known association between the drug and the development of immunosuppression and lymphoma. We recommended periodic examination of the serum proteins in patients receiving diphenylhydantoin in order to detect development of monoclonal gammopathy.

Epilepsy↗

Aberrant congenital dyserythropoietic anemia with negative acidified serum tests and features of thalassemia in a Kurdish family.

Three siblings of a Kurdish Jewish family with clinical and hematologic findings compatible with congenital dyserythropoietic anemia (CDA) are described. All patients presented with mild anemia, marked hyperbilirubinemia and splenomegaly. The bone marrow morphology and ultrastructure of the normoblasts was typical of CDA type II and there was strong agglutination of the patients' red blood cells by anti-i serum. These patients displayed two features that were not characteristic of CDA type II, namely, the acidified serum lysis test was negative on more than 10 occasions, and high levels of Hb A2 were observed in two siblings. In one of the siblings, abnormal globin-chain synthesis was found and alpha-chain production exceeded beta-chain production, as in beta-thalassemia minor. In the light of the above findings, our patients are perhaps best classified as having aberrant CDA with features of thalassemia.

Adult↗