[A case of successfully treated cyclic amegakaryocytic thrombocytopenic purpura].

The case of a patient with cyclic, acquired, periodically amegakaryocytic thrombocytopenia is described. On the base of a seemingly typical megakaryocytic thrombocytopenia ITP was diagnosed at first. He did not respond to steroid, therefore splenectomy was performed.

[Fatal thrombotic microangiopathy in the mother and fetus].

The appearance of thrombotic microangiopathy (thrombotic thrombocytopenic purpura, haemolytic uraemic syndrome) could have been documented in a 23 years old pregnant woman, who had been treated previously for immune-thrombocytolytic purpura. The disturbing anamnestic data caused significant delay in correct diagnosis and in starting of fresh-frozen plasma therapy, so the woman and her fetus (in utero) had been died. The specific histological microangiopathic lesions could have been well documented by the autopsy of the mother, however no such alterations could have been detected in the fetus and placenta.

[Subcutaneous heparin-calcium therapy: clinical and laboratory experience].

Fifty patients with acute deep vein thrombosis, pulmonary embolism, acute myocardial infarct and priapism were treated with subcutaneous calcium heparin. Laboratory control was based on the examination of APTT. On the first day of heparin therapy only 52% of patients's APTT was 1.

[Hairy cell leukemia].

Clinical experiences on seven patients suffering from hairy cell leukaemia are presented. The disease was most often identified at age above 50 and was more frequent in men. Splenomegaly and infiltration of bone marrow or spleen with tartrat resistant acid phosphatase positive lymphoid cells were detected in all cases.

[Thrombotic microangiography (thrombotic thrombocytopenic purpura and hemolytic uremia syndrome)].

Thrombotic thrombocytopenic purpura and haemolytic uraemic syndrome have quite uniform or identical histopathological features and share very similar clinical characteristics, so thrombotic microangiopathy may be used as a common descriptive term of the two syndromes. During the past one and a half year five patients with thrombotic microangiopathy, receiving plasma therapy were observed by the authors. Owing to the early, correct diagnosis and plasma administration four patients had been recovered from the disease.

Cyclosporin treatment of a woman with acquired haemophilia due to factor VIII:C inhibitor.

A 47 year old woman is reported who had life-threatening bleeding due to the spontaneous development of factor VIII:C inhibitor. Cyclosporin combined with prednisone resulted in a full recovery and complete elimination of antibody even when other therapeutic facilities failed to be effective.

Retrovirus-neutralizing antibodies in AML and AMMoL patients: stage-specific distribution.

Plasma samples of patients with AML or AMMoL were tested for antibodies reacting with gp70 antigens of BaEV and GaLV as well as for antibodies neutralizing BaEV or GaLV. Both frequency and titer values of antibodies were higher in remission than in blastosis. Neutralizing activity could be detected only in those plasma samples which contained antibodies to the appropriate gp70 antigen.

Congenital factor XIII deficiency with multiple benign breast tumours and successful pregnancy with substitutive therapy. A case report.

A 34-year-old woman with congenital factor XIII (FXIII) deficiency and multiple connective tissue tumours is reported. The subunit a of FXIII was totally absent in her plasma, platelets and histiocytes of breast fibroadenomas and considerably reduced in the monocytes (below 5%). The plasmatic level of subunit b was also reduced (25%).

Cytochemical studies in the blastic transformation of chronic granulocytic leukaemia.

The cytochemical features of blast cells were studied in 45 patients with blastic phase of chronic granulocytic leukaemia. Various degrees of Sudan black B positivity was characteristic of myeloblastic transformation (23 patients), while in the medullary blast cells of nine patients with myelomonocytic transformation the alpha-naphthyl-acetate esterase showed intensive activity. In two cases the demonstrability of beta-thromboglobulin and factor VIII-related antigen in blast cells showing otherwise PAS, acid phosphatase and alpha-naphthyl-acetate esterase activity referred to megakaryocytic transformation.

Cytotoxic activity of lymphocyte subpopulations against autologous tumour cells in patients with myeloid leukaemias and preleukaemic disorders.

Autologous lymphocyte populations from different phases of chronic granulocytic leukaemia (CGL), acute myeloid leukaemia (AML) and preleukaemic disorders were compared for cytotoxic activity. 51Cr-release tests showed that T lymphocytes from the quiescent phase of CGL and from the remission phase of AML exerted cytotoxic activity against autologous tumour cells. Such activity was also found in patients with potentially preleukaemic haematological disorders characterized by cytopenia, but not in polycythaemia vera patients.

Interferon production in myelo- and lymphoproliferative diseases. I. Spontaneous interferon production in acute and chronic leukaemias.

Blood plasma interferon (IFN) of patients with acute myeloid (AML) and chronic granulocytic (CGL) and with acute and chronic lymphoid leukaemia (ALL and CLL) was measured by bioassay and characterized by neutralization with anti-human-IFN-alpha treatment. Elevated IFN-alpha level (60-125 IU/ml) was found in the quiescent phase of CGL as compared to the control samples (15 +/- 10 IU/ml). No significant differences could be found between plasma IFN of patients suffering from the blastic crisis of CGL and control persons.

Thrombosis promoting changes in chronic liver diseases.

Causes of haemorrhagic tendency in liver disorders have been widely studied. Deficiency of procoagulants is the best explanation for it. Not seldom a thrombotic tendency or even overt thrombosis occurs and may be satisfactorily explained.