Immune thrombocytopenia (ITP) has a favorable prognosis in children. Only a small number of children go on to develop chronic ITP. However, at the time of diagnosis, it is not possible to predict the course of the disease. In order to determine prognostic factors that could predict the disease course at diagnosis, we retrospectively evaluated various clinical variables in 103 pediatric patients with newly diagnosed ITP at our institution from 1995 to 2001. Sixty-eight (66%) patients had a mean platelet volume (MPV) of <8 fL on admission. Of 72 patients who had a follow-up period of at least 6 months, 54 (75%) achieved a durable remission within 6 months and 18 (25%) developed chronic ITP. In univariate analysis, a low admission MPV (<8), history of viral prodrome, and a low admission platelet count (<10 · 10 9 /L) predicted for a favorable outcome. Age and sex did not correlate with remission. In multivariate analysis, a low admission MPV and a history of a viral prodrome were the only independent factors correlated with a durable CR. The adjusted odds ratio for achieving a durable remission was 8.9 (95% CI: 1.54-51.8) for history of a viral prodrome and 14 (95% CI: 2.52-83.3) for low admission MPV value. In conclusion, our study showed that a majority of the children with newly diagnosed ITP presented with a low MPV value. A history of viral illness and a low admission MPV were found to be independent prognostic variables that predicted for the achievement of a durable CR in childhood ITP. Am.

Sickle cell disease is characterized by chronic hemolytic anemia and vaso-occlusive painful crisis. The vascular occlusion in sickle cell disease is a complex process and accounts for the majority of the clinical manifestations of the disease. Abdominal pain is an important component of vaso-occlusive painful crisis and may mimic diseases such as acute appendicitis and cholecystitis. Acute pancreatitis is rarely included as a cause of abdominal pain in patients with sickle cell disease. When it occurs it may result form biliary obstruction, but in other instances it might be a consequence of microvessel occlusion causing ischemia. In this series we describe four cases of acute pancreatitis in patients with sickle cell disease apparently due to microvascular occlusion and ischemic injury to the pancreas. All patients responded to conservative management. Acute pancreatitis should be considered in the differential diagnosis of abdominal pain in patients with sickle cell disease. Am. J. Hematol. 73:190-193, 2003.

Purpose of Review We aim to review the most recent findings in the use of NK cells in childhood cancers. Recent Findings Natural killer cells are cytotoxic to tumor cells. In pediatric leukemias, adoptive transfer of NK cells can bridge children not in remission to transplant. Interleukins (IL2, IL15) can enhance NK cell function. NK cell-CAR therapy has advantages of shorter life span that lessens chronic toxicities, lower risk of graft versus host disease when using allogeneic cells, ability of NK cells to recognize tumor cells that have downregulated MHC to escape T cells, and possibly less likelihood of cytokine storm. Cytotoxicity to solid tumors (rhabdomyosarcoma, Ewing’s sarcoma, neuroblastoma) is seen with graft versus tumor effect in transplant and in combination with antibodies. Challenges lie in the microenvironment which is suppressive for NK cells. Summary NK cell immunotherapy in childhood cancers is promising and recent works aim to overcome challenges.

A 14-year-old girl had an abdominal mass with the characteristics of an ovarian germ cell tumor on computed tomography scan. The mass, arising from the left ovary, was completely resected and found to be osteosarcoma arising from a mature cystic teratoma. A metastatic lesion in the abdomen did not respond to 2 courses of cisplatin, doxorubicin, ifosfamide, and high-dose methotrexate, and was resected. Seven months after completion of chemotherapy, there were simultaneous local recurrence and lung metastases. Previously, 10 cases of ovarian osteosarcoma have been reported in the literature: 5 were primary osteosarcoma of the ovary, 4 were associated with teratomas, and 1 was part of a malignant mixed mesodermal tumor of the ovary. Of the 10, there are only 2 long-term survivors, both of whom were treated with adjuvant chemotherapy following complete resection.