PubMed · 12612185
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Janna M Journeycake, George R Buchanan. 2003. Coagulation disorders.. https://doi.org/10.1542/pir.24-3-83
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We report a case of juvenile xanthogranuloma (JXG) having progressive pancytopenia for 6 months until the proliferating skin lesions. A 2-month-old infant presented recurrent fever, anemia, and hepatosplenomegaly mimicking hemophagocytic lymphohistiocytosis (HLH) or juvenile myelomonocytic leukemia (JMML). At 8 months of age, the biopsy of a growing papule on the elbow made the diagnosis. Bone marrow (BM) specimens showed clustering foamy cells including hemophagocytosis by histiocytes. Treatment with etoposide followed by vinblastine plus prednisolone (PSL) therapy improved the disease. Although JXG is a benign non-Langerhans cell histiocytosis, the multisystem-visceral form should be considered as a potential aggressive disease when associated with BM failure in early infancy.
BACKGROUND: Maternal prothrombotic disorders may contribute to stroke in the fetus before and during birth. Many of the mothers of children with perinatal stroke have no previous history of pathologic thrombosis. OBJECTIVE: To determine if finding the Factor V Leiden mutation, prothrombin 20210 G-A gene defect, or methylene tetrahydrofolate reductase C677T mutation in an asymptomatic mother of a child with perinatal stroke would affect that mother's ability to obtain health insurance. METHODS: 1) The authors reviewed the literature on genetic prothrombotic risk factors and health insurance. 2) The authors surveyed the 17 largest insurance carriers in Indiana to find if diagnosing genetic prothrombotic risk factors in asymptomatic mothers of children with perinatal stroke would affect the mothers' health insurance status. RESULTS: Three articles on genetic prothrombotic risk factors and insurance were identified. Twelve of 17 insurance companies responded to our survey; three had policies on genetic testing. Most companies refused to provide clear, useful information on their policies regarding these risk factors. CONCLUSIONS: The authors are currently unable to counsel their patients' families on the long-term insurance implications of screening for genetic prothrombotic risk factors. The insurance implications of diagnosing healthy women with genetic prothrombotic risk factors need further study.
We present the results of the Joint Task Force on Rational Management of Life Threatening Haemorrhage (LTH) at Rigshospitalet, Copenhagen University Hospital. A standardised treatment algorithm for LTH due to trauma, surgery, or birth is presented. The key feature is balanced transfusion therapy with an "emergency transfusion package" (5 units of red blood cells plus 5 units of fresh-frozen plasma plus 2 units of platelets). The Blood Bank services, guides, monitors and delivers the emergency transfusion packages in accordance with the algorithm.