[Unusual features in Glanzmann thrombasthenia; proliferation of B-lymphocytes, lead poisoning].
Four case histories are reported: 1. A 37-year-old woman suffering from Glanzmann's thrombasthenia has been regularly seen since 1955. Characteristically (and in contrast to the first description by Glanzmann!) persistently prolonged bleeding times were noted. Clot retraction is severely diminished and the platelets fail to aggregate upon various stimuli. (Platelet agglutination upon addition of ristocetin to platelet rich plasma is normal.) The diagnosis of thrombasthenia was confirmed by demonstration of a deficiency of the membrane glycoprotein IIb/IIIa complex. In recent years the patient has become refractory to platelet transfusion therapy, a response shown to be due to antibodies against GPIIb/IIIa in the plasma. Spontaneous bleeding tendency has appeared to improve over the years. 2. Two patients with proliferation of B-lymphocytes are presented. a) Splenomegaly and an increase of B-lymphocytes in the peripheral blood were detected in a 45-year-old asymptomatic man. DNA analysis suggested that polyclonal proliferation of B-lymphocytes was present. Diagnostic considerations are discussed. b) In a 46-year-old male patient with subacute aleukemic leukemia of a pre-B-cell type diagnosed in 1981, the disease showed an unexpectedly benign course: after initial mild chemotherapy the patient has remained in a stable condition while off cytotoxic treatment for the last two years. Nevertheless, besides anemia necessitating regular transfusions, persistent agranulocytosis is present which is not explained by bone marrow infiltration. In vitro experiments suggest suppression of myelopoiesis by cellular interaction with leukemic cells or a deficiency of growth factors causing agranulocytosis. 3. An 81-year-old man showed signs and symptoms of lead intoxication which proved to be due to oral ingestion of a lead-containing ointment.
Bucher
U
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Hämatologisches Zentrallabor, Universität Bern.
D000328:Adult; D000368:Aged; D000380:Agranulocytosis / Q000097:blood; D001402:B-Lymphocytes; D001760:Bleeding Time; D001791:Blood Platelet Disorders / Q000097:blood*; D002455:Cell Division; D003018:Clot Retraction; D005260:Female; D006801:Humans; D007855:Lead Poisoning / Q000097:blood*; D008297:Male; D008562:Membrane Glycoproteins / Q000172:deficiency; D008875:Middle Aged; D010974:Platelet Aggregation; D015452:Precursor B-Cell Lymphoblastic Leukemia-Lymphoma / Q000097:blood*; D013915:Thrombasthenia / Q000097:blood*
D008562:Membrane Glycoproteins
Ungewöhnliches bei Thrombasthenie Glanzmann; Proliferation von B-Lymphozyten, Bleivergiftung.