Clinical Course of Two Children with Unstable Hemoglobins: The Effect of Hydroxyurea Therapy

Figures & data

Figure 1. Laboratory parameters before and during HU treatment of patient with Hb Volga (left panels) and Hb Köln (right panels). Start of HU therapy is indicated by an arrow in the top panels. Period of cholelithiasis (1), pneumonia (2), and Parvo B19 induced aplastic crisis, requiring red blood cell transfusions (3), are indicated with numerals for the patient with Hb Volga. Percentages of Hb Köln are shown by a dashed line with open circles. Hb Volga was not detectable by IEF, but a heterozygous presence of the mutation was confirmed by sequencing.

Table 1. Laboratory parameter values for the patient with Hb Volga.

Parameters	Before Therapy Mean (range)	During Therapy^a Mean (range)
Hb (g/dL)	8.8 (7.6–9.3)	9.8 (7.9–10.3)
MCV (fL)	79.0 (78.0–82.0)	104.0 (98.0–108.0)
Reticulocytes (%)	148.0 (104.0–200.0)^c	107.0 (72.0–155.0)
Leukocytes (10^9/L)	8.2 (6.5–13.5)	3.4 (1.9–5.2)
Neutrophilic granulocytes (10^9/L)	3.5 (3.3–3.7)	1.6 (0.9–2.4)
Thrombocytes (10^9/L)	295.0 (159.0–593.0)	106.0 64.0–150.0)
Bilirubin (μmol/L)	180.0 (142.0–224.0)	218.0 (141.0–310.0)^b
Lactate dehydrogenase (U/L)	929.0 (659.0–1197.0)	940.0 (655.0–1201)
Ferritin (μg/dL)	40.0	86.0 (32.0–161.0)
Hb F (%)	19.0^c	24.0 (19.0–31.0)

^a Period of aplastic anemia, requiring RBC transfusions, not included.

^b Period of cholelithiasis not included.

^c Value at age 7 months not included. See Figure 1 for the indication of Hb, reticulocytes, bilirubin and Hb F values during excluded periods.

Table 2. Laboratory parameter values for the patient with Hb Köln.

Parameters	Before Therapy Mean (range)	During Therapy Mean (range)
Hb (g/dL)	12.0 (11.6–12.4)	12.0 (11.3–12.6)
MCV (fL)	88.0 (86.0–89.0)	103.0 (99.0–109.0)
Reticulocytes (%)	44.0 (37.0–50.0)	39.0 (36.0–44.0)
Leukocytes (10^9/L)	8.3 (6.5–10.4)	5.1 (4.9–5.5)
Neutrophilic granulocytes (10^9/L)	7.2 (5.8–8.3)	2.7 (2.5–2.9)
Thrombocytes (10^9/L)	185.0 (14.9–203.0)	140 (109.0–158.0)
Bilirubin (μmol/L)	38.0 (35.0–42.0)	38.0 (36.0–40.0)
Lactate dehydrogenase (U/L)	427.0 (422.0–432.0)	449.0 (392.0–519.0)
Ferritin (μg/dL)	161.0	ND
Hb F (%)	<1.4 (<1.0–1.8)	<1.0
Hb Köln (%)	7.2 (6.2–8.2)	5.3 (6.0–6.4)

ND: not done.