Clinical Features and Molecular Analysis of Hb H Disease in Taiwan

Table 2

Comparison of clinical features between patients with deletional and nondeletional Hb H disease in Taiwan.

	Deletional Hb H disease	Nondeletional Hb H disease	value
	( = 44)	( = 24)
Initial manifestations
Age at diagnosis (year)	1.7–63.4 (median 24.0)	3.0–36.9 (median 10.4)	0.040*
Gender	21 males, 23 females	15 males, 9 females	0.261
Laboratory data at diagnosis
Hb (g/dL)	5.5–11.5 (median 9.1)	4.6–12.1 (median 8)	0.001*
MCV (fL)	43.9–75.1 (median 60)	63.0–78.1 (median 68.8)	<0.001*
MCH (pg)	15.1–28.0 (median 17.5)	16.8–22.4 (median 18.3)	0.568
MCHC (g/dL)	15.3–33.7 (median 29.0)	19.1–29.4 (median 25.9)	0.740
Hb H (%)	0.7–29.7 (median 5.9)	6.3–35.5 (median 12.3)	<0.001*
Follow-up clinical features
History of blood transfusions	10 (22.7%)	14 (58.3%)	0.003*
Splenomegaly	9 (20.5%)	14 (58.3%)	0.002*
Hepatomegaly	3 (6.8%)	7 (29.2%)	0.013*
Jaundice	4 (9.1%)	13 (54.2%)	<0.001*
Gallstones	2 (4.5%)	5 (20.8%)	0.035*
Cholecystitis	2 (4.5%)	0	0.289
Splenectomy	4 (9.1%)	5 (20.8%)	0.172
Cholecystectomy	2 (4.5%)	0	0.289
Thrombotic event	1 (2.3%)	1 (4.2%)	0.659
Leg ulcers	0	0	—
Growth retardation	0	0	—
Delay of pubescence	0	0	—
Serum ferritin level (ng/mL)	40.2–699.0 (median 142.5)	40.3–1619.0 (median 210.7)	0.001*

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