Daisilee H. Berry

An experience with an implanted port system in 66 children with cancer

Totally implanted port catheter systems have a lower incidence of infection and are more easily used in home care that external catheters in adult cancer patients. Experience with this method in children has been limited. During the past 2 years, we have implanted 71 ports in 66 children with cancer. Our experience demonstrates an infection rate (0.15 episodes of bacteremia per 100 patient days) slightly lower than that reported for children with Broviac or Hickman catheters, but not as low as that seen in adults with implanted systems. Patients and families have been extremely satisfied with the devices. Our experience supports further use of implanted systems in children with cancer.

Chromosome abnormalities in familial hemophagocytic lymphohistiocytosis

Familial hemophagocytic lymphohistiocytosis (FHLH) is an uncommon disorder characterized by multiorgan infiltration with phagocytic histiocytes/macrophages. It may be inherited as an autosomal recessive trait, but specific associated cytogenetic abnormalities have not been documented. The authors describe a 10‐week‐old white female without prior family history of FHLH, who fulfilled the histologic and clinical criteria for the diagnosis. In addition, cytogenetic abnormalities, including the presence of double minute chromosomes and occasional loss of chromosomes 7 and 12, were documented in unstimulated peripheral blood cells. These karyotypic findings are usually associated with dyserythropoietic and leukemic states, and have not been described previously in the context of FHLH. It may be useful to do chromosome analyses on unstimulated peripheral blood cultures from FHLH patients before treatment to examine the karyotype of proliferating cells, which may represent the infiltrative histiocytes seen in the disorder.

Paroxysmal Nocturnal Hemoglobinuria Presenting As Recurrent Hemolytic Uremic Syndrome

Paroxysmal nocturnal hemoglobinuria (PNH) may present with acute anemia, thrombocytopenia and, if hemoglobin nephropathy or dehydration is present, azotemia. Thus PNH may be confused with the hemolytic uremic syndrome (HUS). Recurrent episodes, though common in PNH, are unusual in HUS. A positive acid hemolysis test can be used to differentiate between the two diseases.

Thrombotic thrombocytopenic purpura in an asplenic patient with hereditary spherocytosis: failure of plasmapheresis, antiplatelet therapy, and corticosteroids.

Thrombotic thrombocytopenic purpura (TTP) is a severe multisystem disorder characterized by microangiopathic hemolysis, central nervous system and renal dysfunction, and a very poor prognosis. Recently, however, plasma exchange or infusion therapy has proven effective in the majority of patients with TTP. We report a patient who developed TTP several years after splenectomy for hereditary spherocytosis. Despite aggressive therapy with plasmapheresis (PP), plasma infusion, antiplatelet drugs, and corticosteroids, the patient had progression of TTP that eventually resulted in his death. The occurrence of TTP in an asplenic patient with an intrinsic red cell disorder, a previously unreported association, may predict a poor prognosis.

Comparison of prednisolone, vincristine, methotrexate and 6‐mercaptopurine vs. 6‐mercaptopurine and prednisone maintenance therapy in childhood acute leukemia: A southwest oncology group study

The study was designed to compare the relapse rate and toxicity of intermittent prednisone, oncovin (vincristine), methotrexate, and 6‐mercaptopurine (POMP) vs. 6‐mercaptopurine daily and a prednisone pulse every three months (PIP) for maintenance therapy in acute leukemia. Children with acute lymphoblastic, acute undifferentiated, or acute stem‐cell leukemia were stratified on the basis of initial leukocyte count and age, then randomly assigned to POMP or PIP maintenance therapy. All patients received cranial irradiation and intrathecal chemotherapy. Of the 67 patients receiving POMP maintenance, 20 (30%) remain in continuous remission. The median length of continuous remission achieved with POMP therapy was 49 weeks. Of 80 patients receiving PIP maintenance, 25 (31%) remain in remission. The median length of continuous remission for PIP was 62 weeks. Of the possible prognostic factors evaluated, the only significant factor was the prognostic grouping base in age and initial leukocyte count. Prognostic group I contained the patients with the longest duration of remission. The POMP regimen was associated with a higher incidence of toxic reactions, frequently causing therapy interruption. The results for both regimens as studied are inferior to those for 6‐mercaptopurine‐methotrexate maintenance regimens as reported by others.