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A 21 Trisomy, IUGR with clinical transient abnormal myelopoiesis (TAM) but No blasts detected in the blood.

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Any experience with a case of A 21 trisomy, with clinical manifestations of transient abnormal myelopoiesis (TAM) but no blasts detected in the blood?

Experiencing a case of late preterm, 21 trisomy, IUGR, serologically -ve for HSV, CMV and Parvovirus infections, anemia, hepatomegaly, elevated transaminase by 10 folds, serum T. bilirubin reaching 15 mg / dl and ascites.

Did this scenario justify treating with low-dose cytarabine?

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Treatment in TAM cases is indicated only in the presence of Life threatening complications:

  • Signs of hyper viscosity → blast count > 100,000/uL,

  • Hepatosplenomegaly causing respiratory compromise

  • Heart failure (ejection fraction - 47% or shortening fraction - 27%) not directly the result of a congenital heart defect

  • Hydrops fetalis

  • Renal or hepatic dysfunction

  • Disseminated intravascular coagulation with bleeding.
    COG trial: Adapted from Gamis et al, 2011

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@Radhika Maddali thank you, yes you are correct, These are the indications and the patient developed liver dysfunction HSM, ascites in the 1st week of life as stated (these manifistations could justify useing Cytarabine treatment, if we had detectsble blast cells in the blood sample).

Later on developed thrombocytopenia and coagulopathy.

The main idea of concern and debate was at this stage could we treat with cytarabine in the absence of blast cells in the blood? Can we justify it?

2nd week of life respiratory distress developed requring nCPAP with FIO2 of 0.3 to 0.35.

  • 2 weeks later...

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