A 41-year-old man presented with rhabdomyolysis and sepsis while the peripheral blood smear showed a pseudo-leukemic picture of plasma cells. After starting supportive therapy, the morphologic finding disappeared within 24 h.

We report a retrospective survey of 35 patients (18 males and 17 females) with B-Prolymphocytic leukemia (B-PLL) followed for a median of 63 months. Twelve patients fulfilled Galton's original clinical and hematological criteria, presented with prominent splenomegaly and hyperleukocytosis and showed rapid progression soon after diagnosis. Twelve cases with gradually increasing spleen size and prolymphocyte count had an indolent course. Seven of this group are alive 68 to 164 months after diagnosis, whereas five died from causes unrelated to PLL. Eleven patients who never developed impressive leukocytosis had a variable prognosis. In the group of 17 patients treated with chlorambucil and prednisone (CP) or cyclophosphamide, vincristine, prednisone (COP) 8 achieved a partial remission (PR) with a median response of 32 months. In the group of six cyclophosphamide, doxorubicin, vincristine, prednisone (CHOP) treated patients one achieved a complete remission and two PR (median response was maintained for 30 months). Three patients treated with 2CdA achieved good PR. Six patients remained untreated. Median survival was 65 months and the probability of overall survival for 3, 5, and 10 years was 63%, 56% and 35%, respectively. Anemia < 11 g/dl and lymphocytosis > 100 x 10(9)/l were predictors of shorter survival in this group of patients. Age over 70, gender, B-symptoms at presentation, spleen size, thrombocytopenia, low IgG and complement levels, presence of paraproteinemia and the pattern of bone marrow infiltrate were not significant. Our findings show that all B-PLL may not have such a poor prognosis as described in earlier reports. The existence of prior symptoms evolving gradually after years to obvious PLL and cases with mild prolymphocytosis could possibly lead to underdiagnosis of the entity. Identification and follow-up of such cases may suggest a different natural history, variable prognostic features and different survival curves for B-PLL patients. In the light of the above, we suggest that the therapeutic approach for B-PLL should always relate to the severity of the disease.

No abstract

In an autopsy material of 29 cases of the hypoplastic left heart syndrome coronary fibroelastosis was found in 1 case, endocardial fibroelastosis in 8 cases. Figures for 10 cases of the hypoplastic right heart syndrome were 6 cases of coronary fibroelastosis and 1 case of endocardial fibroelastosis. Age ranged from stillborn up to 11-1/2 months. Coronary and endocardial fibroelastosis seemed to be mutually exclusive localizations of congenital fibroelastosis since in our material they did not occur together in the same hearts. In hypoplastic right hearts coronary fibroelastosis was either restricted to the right coronary artery (right circumflex and posterior interventricular branch), or it was found also in the left coronary artery (anterior interventricular branch), with the most serve affections always being situated in the right one. In the only case of coronary fibroelastosis among the hypoplastic left hearts the condition was limited to the anterior interventricular branch of the left coronary artery which communicated with the hypoplastic left ventricle by a fistula. Coronary fibroelastosis was exclusively found in branches supplying the hypoplastic right ventricle and/or in a branch connected by a fistula to the hypoplastic left or right ventricle. Endocardial fibroelastosis was generally found in hypoplastic left ventricles with either no outflow or with severe outflow obstruction. A theory concerning the aetiology of both coronary and endocardial fibroelastosis of the hypoplastic ventricles is proposed. It is argued that development of fibroelastosis may in both localizations be caused or favoured by the coincidence of two factors: abnormal haemodynamic conditions and poor oxygenation of blood and tissues. Observations made in a reference material of 35 hypoplastic left and 24 hypoplastic right hearts were in accordance with this view.

We describe a female patient who presented at pregnancy with leucopenia and was found to suffer from both fragile X syndrome [Fra(X)] and myelodysplastic syndrome with cytogenetic abnormalities in bone marrow cells including 4q+ and deletion D13. To date only four cases of Fra(X) syndrome with malignant tumours (one haematological), all in male patients, have been reported. We believe that the occurrence of the myelodysplastic syndrome in this patient could be more than coincidental.