Discussions By Condition: I cannot get a diagnosis.

bleeding tendency for D.D.

Posted In: I cannot get a diagnosis. 0 Replies
  • Posted By: Anonymous
  • December 27, 2009
  • 10:21 PM

Case summary
Bleeding tendency infant
Name : yassin ayman
Age: 1,5 year
Sex: male
-ve consanguinity
Complain : bleeding per nose , vomiting of blood , bleeding per-rectum , bleeding with micturation.
History : the condition started 5 months ago by diarrhea more than 13 motions daily associated with vomiting and fever , sought for medical advice diagnosed as gastroenteritis treated with ORS and anti-biotic without response , admitted at tanta hospital with dehydrations and treated with IV Fluids .
Later on and within 2 days he developed bleeding per-rectum , epistaxsis , associated with high grade fever ,CBC show normocytic normochromic anemia without thrombocytopenia , stool culture done and he was given Anti-biotic according to the result but without good response , 1 day later the general condition deteriorate
In the form of febrile convulsions and DCL.
The infant referred to the ICU and diagnosed as DIC, as he was presented with epistaxsis hematemsis hematuria bleeding per-rectum and FDS > 20, D-dimer +ve
But without thrombocytopenia and normal coagulation profile PT,PTT, PC,INR ,APTT,BT . Treated with IVIG for 2 days without response so referred to CAIRO UNIVERSETY pediatric hospital.
At hematology department the infant was presented with the following :
Frank hematuria , bleeding per-rectum ,epistaxsis and bleeding from ears with average general condition and well performance status ?!!!
We start to give him plasma daily for 2 weeks so all bleeding from all orifices stopped except hematuria persist even on regular daily plasma transfusion .
Nephrology consultation and pelvi-abdominal ultra-sound revealed nothing abnormal within the urinary system.
We stop plasma for 2 days in order to reevaluate the coagulation profile and it was normal but the infant start hematemsis ,bleeding per-rectum , epistaxsis and deterioration in the general condition , so we gave him plasma again daily and the same occur all bleeding from all orifices stopped except hematuria .
We gave him cryo for 3 days but bleeding persist per rectum ,nose and hematemsis
Again on plasma all bleeding stopped except from all orifices except hematuria ,
The same occur with platelet transfusion.
CBC revelaed normocytic normochromic anemia only and normal coagulation profile but platelet function test revealed defect in platlet agreggations with adrenaline and ADP but normal with ristocetin.
During all this time blood was given twice only when we stop plasma for 2 days.
Till today the infant presenting with frank hematuria not responding to plasma cryo or platelet.!!!!!

lab work up :
coagulation profile normal
cANCA +ve
anti-cardiolipin IgM +ve
C3,C4 normal
urine analysis : albumin +++ , RBCs more than 100 , pus cells 5-10
platelet function assay : defect in platelet agreggation with ADP and adrenaline

my e-mail : asas123eg@yahoo.com

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