Homozygous protein C deficiency with delayed onset of symptoms at 7 to 10 months.

We report an inbred family with two cases of homozygous protein C deficiency and review 11 other such cases. Both patients presented in the second half of their first year of life with recurrent rapidly disappearing ecchymotic skin lesions, disseminated intravascular coagulation, and venous thrombosis. Successful treatment has been achieved by frequent infusions of plasma or prothrombin complex then maintained with Warfarin. Homozygous recessive protein C deficiency usually presents in the neonatal period with purpura fulminans. Two cases have been described elsewhere which presented in the second decade of life with milder symptoms. The present cases appear to be intermediate in time of presentation and severity of symptoms. We also review the distinction that is now evident between recessive and dominant protein C deficiency.

MOPP therapy in children with Hodgkin's disease.

Between 1979 and 1987, 28 children with Hodgkin's disease were treated with MOPP (nitrogen mustard, Oncovin, prednisone, procarbazine) combination chemotherapy without radiotherapy. Twenty-four were staged clinically. Splenectomy was performed in four only. Staging was as follows: nine (32%) in stage I, five (18%) in stage II, nine (32%) in stage III, and five (18%) in stage IV. Histologic types were lymphocytic predominance in five (18%), mixed cellularity in 15 (54%), nodular sclerosis in seven (25%) and lymphocytic depletion in one (4%). All children achieved complete remission. Two in stages III and IV relapsed and were salvaged with additional chemotherapy and radiotherapy. Twenty-six are in continuous relapse-free remission for periods ranging from 2 to 9 years. The relapse-free survival rate of 92% and survival rate of 100% compares favorably with results obtained using combined modality treatment.

Burkitt's lymphoma in Jordanian children: epidemiological and clinical study.

Twenty-four children with a diagnosis of Burkitt's lymphoma were seen at Jordan University Hospital during a 6 year period (1978-1983). Their median age was 4 years and the male to female ratio was 3:1. A space clustering of cases was evident. Abdominal mass was the commonest presentation being observed in 21 patients, while only two patients presented with jaw masses. Sixteen patients had advanced disease (stages C and D). Chemotherapy was the basic form of therapy given. Twelve children (50%) survived. Advanced stage and poor nutritional status at diagnosis correlated with poor outcome. Our data indicate that Burkitt's lymphoma in Jordanian children presents an intermediate type that shares some similarities with both the endemic (African) and non-endemic types.

Mixed mesodermal tumor of the uterus in a 4-year-old girl.

A case of malignant mixed mesodermal tumor (MMT) of the uterus in a 4-year-old girl is reported. The patient had a polypoid lesion protruding from the vagina which was initially thought to be a sarcoma botryoides. This is one of the youngest cases of MMT on record. A review of MMT in premenopausal women and children is presented.

Improved survival in young children with acute granulocytic leukemia treated with combination therapy using cyclophosphamide, oncovin, cytosine arabinoside, and prednisone.

Seven of 17 children (41%) under 5 years of age with acute granulocytic leukemia (AGL) treated with either cytosine arabinoside-cytoxan (CA-CYT) or Mini-COAP (CA-CYT with vincristine sulfate [VCR] and prednisone) have been in continuous complete remission 4 years or more. CA and CYT were each given in the dosage of 120 mg/m2 intravenously, daily in 3 divided doses, for 4 days. Induction consisted of two courses given at intervals of 2 weeks; during maintenance the courses were repeated at intervals of 4 weeks. In the Mini-COAP regimen, standard 28-day VCR-prednisone therapy was superimposed on CA-CYT induction and 4-day VCR-prednisone pulses were superimposed on CA-CYT maintenance. Transient moderate to severe myelosuppression was frequent; other manifestations of toxicity were mild. Administration of drugs at home was feasible in many instances. Mini-COAP was proved to be an effective therapeutic regimen for young children with AGL and should be considered as initial therapy.