Hematology Case 45

35 year old male

• CBC:

- WBC: 6.42x10³ /uL (Neutrophils: 2.1x10³ /uL - Lymphocytes: 3.5x10³ /uL - Monocytes: 0.6x10³ /uL - Eosinophils: 0.2x10³ /uL - Basophils: 0.02x10³ /uL)

- HGB: 10.2g/dL - MCV: 112 fL -MCH: 40 pG - MCHC: 35.7%

- Platelet: 274x10³ /uL

• Reticulocytic Count: 3.1% - Absolute Reticulocytes: 79.5x10⁹ /uL (R.R.: 50-100)

• LDH: 247 u/L (R.R.: 98-192)

• Vitamin B12: 223.9 pG/mL (R.R.: 240-900)

Peripheral Smear:

Mild macrocytic normochromic anemia. RBC show mild anisopoikilocytosis. Sickle cells and target cells are frequently seen.

HPLC for HGB:

Pattern of Homozygous sickle cell disease.

Elevated HGB F is secondary to Hydroxyurea therapy (Sign of response to hydroxyurea therapy)

Co-inheritance of non-deletional hereditary persistence of fetal haemoglobin is a differential diagnosis.

• Suggested Redding:

(((Haemoglobin F is mildly elevated in homozygotes for β S associated with the Bantu, Benin or Cameroon haplotypes (5 – 7%) but may be more markedly elevated in association with the Senegal haplotype (7 – 10%) and even more in association with the Arab - Indian haplotype (10 – 25%). Furthermore, the haemoglobin F percentage is increased if there is co - inheritance of non - deletional hereditary persistence of fetal haemoglobin, a quite common occurrence. Patients with sickle cell anaemia may be treated with hydroxycarbamide specifically to raise the haemoglobin F percentage and, when effective, the haemoglobin F percentage is, of course, raised further. ))) Barbra J. Bain, et al, Variant Haemoglobins - A giude to identification, 2010, P 33.

(((Sickle cell anemia; A high MCV is usually due to administration of hydroxycarbamide, which may not be known to the laboratory. However the possibility of coincidental vitamin B12 deficiency must not be overlooked, particularly in patients who are being maintained on folic acid))) Barbara J. bain, Haemoglobinopathy Diagnosis, 3rd Edition, 2020, P208

(((The haemoglobin F percentage in sickle cell anaemia is of prognostic significance, the prognosis being more favourable when the percentage is high. The haemoglobin F percentage is increased 2‐ to 16‐fold by hydroxycarbamide therapy))) Barbara J. bain, Haemoglobinopathy Diagnosis, 3rd Edition, 2020, P221