Three months male baby from Yemen complianing of pallor since first month. Condition was started since birth when the baby developed jaundice at 1st day. (He was full term, normal vaginal delivery).
Investigation at 1st day:
• His Blood group O-ve – Mother’s B-G O+ve
• Bilirubin was 23,5 mg/dl – direct 2,03 mg/dl
• Reticulocyes 20%
• Hb = 9,7 g/dl - pcv 31,7%
• WBC = 27,70
o Neutrophil = 26
o Lymphocyte = 65
• Platelet = 125
Bilirubin not respond to phototherapy so Blood exchange was done after it HB was 11,3 g/dl - pcv = 34% - Bilirubin = 8.5 – Direct = 0.74.
After that he was on phototherapy, investigation at 3rd day:
• Hb = 13,8
• Pcv = 43,1
• Wbc = 9
o Neutrophil = 62
o Lymphocyte = 32
MCV = 86,9
MCH=27,8
MCHC=32,1
RDW=15,0
Bilirubin =15 mg/dl
Ritcs=5.5%
o Platelet = 63
With Blood Film:
• RBc normocytic normchromic with some marocytes of few nucleated cells no specific abnormalities .
• WBc Normal count, Normal Morphology.
• PLTs Low count with Normal Morphology.
Platelet transfusion was done and it became = 185 AND continued on phototherapy.
He discharged after 7 days with total bilirubin 10,20 mg/dl – direct 1,1 T. bilirubin repeated after 1 WK 1 mg/dl.
Patient is well active, normal breast feeding in addition he get artificial milk.
He was ok until he was two months when his mother noted that he was pale -
• Hb = 5 g/dl
• Pcv = 15,6
• Wbc = 9,4
• PLT = 213
• MCV = 89
• MCH= 28
• McHc= 32
• RDW = 23,4
Blood Film report:
RBC Normocytic Normochromic with some polychromatic and few spherocytes fragmented and nucleated cells.
WBC Normal.
• Reticulocytes = 8.5%
• Bilirubin = 2
• LDH = 3244
• Direct combs test –ve
• G6PD = 243 (Normal 60-130)
• Osmotic fragility test –ve
• Electrophoresis Normal
• Fe = Normal – Ferrtin = high
Transfusion of 80 cc packed RBc was done (he was 5 kg).
One month later, patient became pale again with Hb 7 g/dl retics = 3.2 and other 80 cc were given.
• By examination of patient there’s only spleenomegaly that confirmed by ultrasound + He was pale.
Chest is clear, Normal heart sound
• No change in urin color
B.W = 5,5 kg
What is the possible cause?!!!
What other investigation is needed?
how frequnts to do investigation?
what is the haemoglobin level for blood transfusion?