Hematology Case 63
Presented By Dr Mohammad Moharram
Released on 30-04-2023Patient Data
Female Patient 38 year old
Clinical Data
History: Known patient of homozygous sickle cell disease (HGB electrophoresis was documented on many occasions before). Regularly visits the hospital for follow up. History of cholecystectomy. Frequent blood transfusion; Last time 2 years ago.
Examination: Pale, Tinge of Jaundice, mild hepatomegally.
Related Laboratory Results
CBC:
- WBC: 6.95x103 /uL .
- HGB: 10.6 g/dL - MCV:88.2 fL -MCH: 30.9pG - MCHC:35%
- Reticulocytic count%: 7.1% (R.R.: 0.5-2) - Absolute Reticulocytic count: 252x109 /uL (R.R.: 50-100)
- Platelet: 598 x103 /uL
Total bilirubin : 32 umol/L (R.R.: 5.1-20.5) - Direct bilirubin: 5.5 umol/L (R.R.: 1-5.1)
LDH: 321 u/L (R.R.: 98-192).
Serum Ferritin: 171 ng/mL (R.R.: 13-158)
Vitamin B12: 79 pg/mL (R.R.: 180-914) - Serum folate: 54.4 nmol/L (R.R.: 7-30).
Case Picture(s) / Photo(s)
Images Comment / Findings
HPLC technique for HGB revealed ;
HGB F: 6.1% - HGB A2: 4.4% - HGB S: 89.5%
Homozygous Sickle Disease with elevated A2
Final Diagnosis
Elevated HGB A2 with normal RBCs indices together with decreased serum vitamin B12 suggest acquired HGB A2 elevation.
Case of Homozygous sickle cell with acquired HGB A2 elevation.
It is recommended to repeat HGB electrophoresis (HPLC) after treatment of Vitamin B12 deficiency.
We recommend the patient to do thyroid function tests, malaria test & viral markers for HIV.
Additional Note (Click on item):
We excluded Sickle cell / B thalassemia because of:
1- Patient did HGB electrophoresis (HPLC) many times before in our facility & in other facilities. All of them had non significantly elevated HGB A2.
2- Normal RBCs indices (Normal range MCV, MCH, MCHC).
3- Presence of a defined cause for acquired HGB A2 elevation. (Low vitamin B12)
Acquired causes of increased or decreased HGB A2 %:*
* Bain B.J. , 2020. Haemoglobinopathy Diagnosis. 3rd edition. Blackwell Publishing Ltd. Page 333.