Acute myelomonocytic leukaemia and acquired haemophilia A with severe cutaneous haemorrhage.

A case of severe cutaneous haemorrhage due to thrombocytopenia in combination with an acquired haemophilia A is reported. The thrombocytopenia was due to acute myelomonocytic leukaemia (FAB M4). A factor VIII:C specific anticoagulant was also found.

Trisomy of chromosome 22 in acute myelomonocytic leukemia.

A patient with acute myelomonocytic leukemia type M4, with a trisomy 22 as the only chromosomal abnormality is reported. All six previously published cases with this anomaly had acute myeloid leukemia. The subtype was AMMoL in five patients, and the subtype of the sixth one was not indicated.

Evidence of fibrinogen breakdown by leukocyte enzymes in a patient with acute promyelocytic leukemia.

On daunomycin treatment of a patient with promyelocytic leukemia, leukocyte elastase appeared in large amounts in the patient's blood. Also, the plasma fibrinogen was found to be partially degraded to early, X-like, fibrinogen degradation products. These early fibrinogen fragments were isolated and showed a low anticoagulant activity in a thrombin time test.

The in vitro growth pattern of human bone marrow in methylcellulose stimulated by different concentrations of conditioned medium.

The proliferation of human bone marrow in methylcellulose stimulated by various concentrations of conditioned medium (CM) and observed at various intervals was studied. The growth kinetics of granulocytic aggregates was found to differ from monocytic clusters and colonies. Granulocytic aggregates showed a consistent and reproducible dose-response relationship; at day 7, the maximum number of granulocytic aggregates was found at 4% CM.

Immunological investigations in aplastic anemia patients.

In 53 patients with aplastic anemia (AA) a number of parameters concerning immune responsiveness were analyzed. Severe monocytopenia and B-lymphocytopenia was detected in most patients, especially in those with diffuse lymphocyte infiltration in the bone marrow. T-inducer (OKT4)/T-suppressor (OKT8) ratios were normal.

The factor VIII ratio in normal and pathological pregnancies.

The factor VIII ratio was measured in 102 pregnant women (43 uncomplicated pregnancies, 19 with pre-eclampsia without fetal growth retardation, 8 pre-eclampsia complicated by fetal growth retardation, 12 with only fetal growth retardation and 20 with gestational diabetes). The pathological pregnancies showed a statistically significant elevation of the factor VIII ratio compared to the normal pregnancies. The group of pre-eclampsia complicated by fetal growth retardation showed the most marked increase of the factor VIII ratio.

Teratoma of the lung in a hemophilic patient.

We describe a patient with hemophilia A and primary malignant teratoma of the lung. Hemothorax and pseudotumor in hemophilia are rare disorders and can raise many differential diagnostic problems.

Busulphan aplasia in rabbits: a model for human aplastic anaemia.

Bone marrow histology plays a crucial role in the clinical diagnosis of aplastic anaemia. The nature of the disease means that few studies are available on the histological changes which occur in the early stages of the development of aplasia. We describe here an animal model which may have some relevance in this respect.

Anti-thymocyte globulin treatment for aplastic anemia.

20 patients with severe aplastic anemia were treated with anti-thymocyte globulin (ATG), 6 of them in combination with haplo-identical bone marrow. 7 patients (35%) showed a good clinical response within 6 months; they were off transfusions and had greater than or equal to 0.8 x 10(9)/l neutrophils.

Inhibition of CFUE and BFUE by mononuclear peripheral blood cells during chronic benzene treatment in rabbits.

Peripheral blood cells from untransfused rabbits that were treated with a moderate dose of benzene during 2 months were cocultivated with autologous and normal bone marrow. In a significant number of experiments, CFUE and BFUE growth of both autologous and normal bone marrow was suppressed. Further experiments showed that this inhibition was associated with adherent mononuclear peripheral blood cells.

Anti-thymocyte globulin (ATG) can eliminate platelet refractoriness.

Of the 14 patients with aplastic anaemia treated in our hospital with anti-thymocyte globulin (ATG), four were refractory to random platelets before therapy due to the presence of leucocyte antibodies. In contrast to the ten nonrefractory patients in whom no success was obtained, three of the four refractory patients showed haematological improvement after ATG. Additionally, two patients could be substituted again with random platelets.

111Indium-chloride bone marrow scintigraphy in aplastic anaemia.

Bone marrow scintigraphy, using 111Indium-chloride, was performed in 24 patients with acquired aplastic anaemia to investigate: (1) a possible relationship between bone marrow scintigraphy and peripheral blood cell values, (2) a possible relationship between scintigraphy and histology of the bone marrow and (3) the ability to distinguish various aplastic anaemia subtypes with bone marrow scintigraphy. For this purpose a semi-quantitative scoring of scintigraphic results was used. Only a weak correlation was found between the radionuclide studies and blood counts.

Scintigraphic aspects of 111In-chloride bone marrow scintigraphy in aplastic anaemia.

In 25 patients and 11 follow-up studies the clinical usefulness of bone marrow scanning with 111In-chloride in aplastic anaemia is evaluated. Semi-quantitative scanning of bone marrow scan results and the presence of bone marrow extension have proven to be helpful in monitoring the disease and determining aetiological factors. This study shows that 111In-chloride is able to demonstrate regional difference in the bone marrow status of the patient suffering from aplastic anaemia, thereby offering a useful alternative for iron radionuclides.

Extreme potassium loss in a patient with severe graft versus host disease.

A 19-year-old male, suffering from post-hepatitis aplastic anaemia, was transplanted with bone marrow cells from his HLA-identical, MLC non-reactive brother. Haematological recovery ensued, but the patient also developed grade IV graft-versus-host disease (GVHD). In addition to involvement of skin, liver and gut, the kidney seemed affected by GVHD since the patient has hypokalaemia and severe hyperkaluria.

Megakaryoblastic leukaemia (acute myelofibrosis): a report of three cases.

Three patients with megakaryoblastic leukaemia are described. All three presented with pancytopenia, a few blast cells in the peripheral blood and absence of overt hepatosplenomegaly. In two of them bone marrow aspiration yielded a dry tap.

Treatment of aplastic anaemia by antilymphocyte globulin with or without marrow infusion.

Forty-one patients, suffering from severe aplastic anaemia were treated either with ALG alone (27 patients) or ALG followed by infusion of allogeneic bone marrow (14 patients). Eighteen patients (67 per cent) are presently alive after ALG alone at over 100 to over 550 days. Fourteen (52 per cent) showed sustained improvement of haematopoiesis, two are alive without change, one recovered autologous haematopoiesis after cyclophosphamide conditioning and transfusion of HLA identical marrow and one is lost to follow-up.