Marked eosinophilia in an immunosuppressed patient with strongyloidiasis.

Strongyloidiasis in immunocompromised patients is typically associated with a fulminant clinical course and little eosinophilia. We describe an immunocompromised patient with strongyloidiasis in whom the infection was only minimally symptomatic in association with a dramatic increase in eosinophil count.

Molecular analysis of a CML patient with a long duration of chronic phase before and after lymphoid blast crisis.

A patient who was diagnosed with chronic myeloid leukemia remained in chronic phase for 14 years before progressing into a lymphoid blast crisis in 1983. The acute phase was successfully treated, and the patient has remained in an indolent chronic phase to date. Cytogenetic and molecular analysis during this second chronic phase confirm the presence of the Philadelphia chromosome and its transcribed BCR-ABL mRNA. The breakpoint within M-bcr occurred in the 3' portion of the region and expressed a hybrid joining the b3 exon of BCR to the a2 exon of ABL.

Rapid Remission Induction and Improved Disease Free Survival in Acute Myeloid Leukaemia Using Daunorubicin, ARA-C, and CCNU.

Thirty-four patients with acute myeloblastic leukaemia were treated with DAC, a schedule containing the nitrosourea CCNU (lomustine) 200 mg/m2 given on day one of treatment, together with a standard "3 + 7" remission induction schedule of daunorubicin (DR) and cytosine arabinoside (Ara-C). The results were compared with an historical control group of 24 patients who received 3 + 7 remission induction (DA). The DAC patients were older (median age 55 years) compared with the DA patients (median age 42 years), and had a higher frequency of poor prognosis features including secondary AML and prior myelodysplasia (11/34 DAC patients versus 1/24 patients receiving DA). Overall remission induction was the same for both groups (79%), but 89% of DAC patients who achieved remission did so with one course, compared with 37% of DA patients. The cytopenic phase following a single course of DAC was only slightly longer than that of a single course of DA (26 days vs. 19.5 days). DAC also gave a higher three year actuarial survival than DA (34% vs. 11%), and a lower relapse probability (44% vs. 74%). These results support the hypothesis that chemotherapy for AML may be favoured by including agents such as CCNU, which are active against both non-cycling and cycling leukaemic stem cells, in remission induction schedules.

Fatal familial haemolytic anaemia.

A 19-year-old man, mentally handicapped but physically well, died within 2 days of onset of an acute episode of gastrointestinal upset and "haematuria." Autopsy and microscopy suggested a haemolytic episode and family studies showed haemolytic anemia due to glucose-6-phosphate dehydrogenase deficiency, which was almost certainly present in the deceased.

Activation of coagulation and fibrinolytic systems following stroke.

When the human blood coagulation and fibrinolytic systems are activated thrombin cleaves fibrinopeptide A (FPA) and plasmin cleaves b beta1 leads to 42 from fibrin(ogen). Elevated plasma concentrations of FPA and B beta 1 leads to 42 are evidence for enhanced thrombin and plasma activities in plasma. We have determined the plasma concentrations of FPA and B beta 1 leads to 42 in patients who have had thrombotic stroke. Patients who were studied immediately following stroke were found to have greatly elevated plasma FPA and B beta 1 leads to 42 levels, but these decreased to the concentrations found in an apparently healthy age-matched control group 1 month after the infarct. In contrast, the plasma concentrations of the platelet release product beta-thromboglobulin (beta TG) were slightly, but significantly, elevated immediately following the stroke and these did not alter with time after the infarct. It is concluded that following thrombotic stroke increased thrombin and plasmin activities are to be found in plasma. These increased protease activities are probably not directly associated with an increased in vivo platelet release reaction and may be useful in deciding which patients are at risk of reinfarction or stroke progression.

In vivo platelet release in myeloproliferative disorders.

The in vivo platelet release reaction in 22 patients with myeloproliferative disorders has been studied by measuring plasma concentrations of the platelet release product beta-thromboglobulin (beta TG). Mean beta TG and mean beta TG: whole blood platelet count ratio were significantly raised in the patient group taken as a whole compared to an age matched control group. No significant increases were observed in the plasma concentrations of thrombin and plasmin sensitive fibrinogen fragments fibrinopeptide A (FpA) and B beta 1-42. The patients were divided into those who had normal, increased or decreased responses to in vitro ADP-induced platelet aggregation. Mean beta TG and the mean beta TG: whole blood platelet count ratio were higher in the increased and decreased responders to ADP than in the normal aggregation group, but the differences in means were not statistically significant. Aspirin given to six patients at a dose sufficient to eliminate the secondary phase of ADP-induced platelet aggregation reduced mean beta TG and the mean beta TG: whole blood platelet count ratio but did not alter mean FpA and B beta 1-42. It is concluded that the enhanced platelet release reaction seen in myeloproliferative disorders is independent of plasma protease activity that arises when coagulation and fibrinolytic systems are activated.

Erythrophagocytosis by acute lymphoblastic leukaemic cells.

Phagocytosis of erthyrocytes and platelets by bone marrow blast cells has been noted in 4 patients in the late relapse of acute lymphoblastic leukaemia (ALL). The underlying mechanism is unclear but prolonged course of the disease seems to be a major factor in the emergence of cells with phagocytic properties.

Portal hypertension in a patient with chronic myeloid leukaemia.

A patient with chronic myeloid leukaemia treated with busulphan for 4-5 years, developed signs of busulphan toxicity and portal hypertension with ascites, oesophageal varices and jaundice. At post-mortem there was minimal leukaemic infiltration but there were alterations in the liver architecture sufficient to explain the portal hypertension. The pathogenesis of the liver changes and their possible relationship to splenomegaly and busulphan toxicity are considered.

Tetraploid cell line in a girl with acute leukaemia.

In a girl with atypical acute leukaemia the malignant cell line in peripheral blood and bone marrow during these relapses was found to be tetraploid. The abnormal cells were more proliferative than those usually present in acute leukaemia. The tetraploid DNA content of the blast cells distinguished them from other cells in blood and bone marrow from this patient and from the blast cells found in other instances of acute lymphoblastic leukaemia.

Bone marrow appearances in reticulum cell sarcoma.

18 patients with malignant lymphoma "histiocytic type" were investigated for the bone marrow involvement. Bone marrow aspirates and needle biopsies were used. Smears and sections of the aspirates were examined. The findings in the sections of both aspirates and biopsies were complementary and far more valuable than the bone marrow smears alone. It is concluded that bone marrow examination is helpful in determining the spread of the disease and should be repeated during the course of lymphoma.