Primary plasma cell leukaemia: immunological and ultrastructural studies in 6 cases

The clinical and laboratory studies of 6 patients with primary plasma cell leukaemia are described. The leukaemic cells had a variable morphology, ranging from lymphoplasmacytic and mature plasma cells to poorly differentiated blasts. The neoplastic plasma cells had a characteristic phenotype: they were positive for CyIg and the McAb OKT10 and Ri-3, and did not express the B-cell antigens Ia, B1 and B4. Ultrastructural studies confirmed the plasma cell nature of the leukaemic cells and showed the presence of a meshwork of cytoplasmic fibrils in 50 to 90% of the neoplastic cells from all cases. The distinct ultrastructural and immunological features of PCL described in this study will help the diagnosis and further characterisation of this disease entity.     

Acute porphyria; two cases with autopsy reports

Two cases of acute porphyria are described with particular reference to the general course of the disease and to the findings demonstrable at autopsy. Both cases were characterized by rather typical exacerbations and remissions of symptoms referable primarily to the nervous system and to the gastrointestinal tract. The familial tendency was demonstrated in the first case. Histological study of tissues revealed the most significant changes in the peripheral nerves in which demyelinization was the outstanding feature. Loss of striations in skeletal muscle accompanied the neurological abnormalities. The identity and source of this toxic agent, as well as an interpretation of the pathogenesis of this type of disturbed metabolism, is still being sought.     

Eisenmenger's complex: report of two cases and review of cases with autopsy study

Two cases of Eisenmenger's complex are described. The total number of such cases reported with pathological study is eighteen. The clinical and pathological findings in these patients are tabulated and discussed. Suggested diagnostic criteria, on the basis of these proved cases, are given.The first case is the third reported having associated with it a patent ductus arteriosus. The second case is the second reported having coarctation of the aorta associated with Eisenmenger's complex. Such associated anomalies are frequent.     

Unusual leukemic presentation of rhabdomyosarcoma: report of two cases with immunological, ultrastructural and cytogenetical studies.

BACKGROUND: Bone marrow infiltration occurs rarely at presentation of rhabdomyosarcoma (RMS) or other childhood solid tumors. This possibility leads to misdiagnosis of leukemia and incorrect therapies might be administered. METHODS: We report two patients presenting with diffuse bone marrows involvement by neoplastic cells. Initial studies were not consistent with a diagnosis of leukemia and the cases were further studied extensively by indirect immunofluorescence, immunocytochemistry, electron microscopy and cytogenetics. RESULTS: In both cases blast cells were large, poorly differentiated, with immunological reactivity to the anti-desmin antibody. Ultrastructural findings of muscular features and chromosomal translocation t(2;13) (q37;q14) further confirmed the diagnosis of rhabdomyosarcoma of the alveolar subtype. This was then confirmed histologically in one patient. CONCLUSION: This study stresses the utility of analyzing cases of morphologically undifferentiated marrow blast cells by various techniques, as well as investigating for different types of both hematological and solid neoplasms.     

Clinical and immunological studies of rotavirus vaccines

The RIT 4237 bovine rotavirus vaccine has served as a useful model for rotavirus vaccination, but the vaccine will not be further developed or tested. The main problem encountered with this vaccine was its poor "take" rate in developing countries. The reasons for this are unclear, and it is not known whether other bovine rotavirus vaccines are more efficacious in this respect. WC-3 bovine rotavirus vaccine will shortly be tested at several sites in developing countries. The rhesus rotavirus vaccine RRV-1 does not appear by itself to be a practical vaccine either. It has induced only moderate protection against human rotavirus serotypes other than the vaccine type. However, the fact that RRV-1 vaccine has induced substantial protection against severe diarrhoea caused by serotype 3 rotavirus, even in young infants, is promising and supports the concept that serotype-specific neutralizing antibodies play a role in protection against human rotavirus disease. Based on this concept, it has been possible to develop reassortant rhesus rotaviruses in which one RNA segment of human rotavirus, which encodes the expression of VP7 antigen, has been incorporated (Kapikian et al., 1986; 1987). Such rhesus-human reassortant rotaviruses, representing serotypes 1, 2 and 4 of human rotavirus and serotype 3 of rhesus rotavirus, can be combined to make a tetravalent vaccine that might induce neutralizing antibodies against each of the main serotypes of human rotavirus. Although its efficacy is unproven, such a combination vaccine is presently regarded as the most promising candidate rotavirus vaccine for the prevention of human rotavirus disease.     

An autopsy case of familial idiopathic dilatation of bilateral atria.

A 59-year-old male patient was followed up for congestive heart failure. Echo cardiogram showed no abnormal findings other than a remarkable dilatation of the bilateral atria. The coronary arteries and left ventricular contraction were normal. Left ventricular endomyocardial biopsy showed no significant abnormal findings. Further, we examined his siblings using dynamic magnetic resonance imaging (MRI) and found that they all also had dilated bilateral atria. After several hospitalizations, the proband died from cardiogenic shock. Pathological findings showed nonspecific change in bilateral atria and ventricles. This is a very rare case of familial idiopathic dilatation of bilateral atria.     

Cytochemical, immunological and ANAE-isoenzyme studies in acute myelomonocytic leukaemia: a study of 39 cases

This study reports the cytochemical, electrophoretic and immunological characteristics of blasts cells from 39 cases of acute myelomonocytic leukaemia (M4). The results indicate considerable cytochemical heterogeneity, particularly with respect to esterase (alpha naphthyl acetate and chloroacetate) activities and suggest that an increased serum lysozyme concentration is a more consistent feature. Investigations with a range of monoclonal antibodies also revealed some differences in expression of monocyte-associated determinants although it is considered that immunological assessments are more consistent than cytochemistry in the detection of monocytic blast cell components. Analysis of ANAE isoenzymes by isoelectric focusing was found to be of particular value in cases where interpretation of ANAE cytochemistry was difficult.