Secondary pulmonary alveolar proteinosis complicating myelodysplastic syndrome results in worsening of prognosis: a retrospective cohort study in Japan

Table 1 Demographic data at diagnosis of MDS in each group classified according to the WPSS

		<WPSS risk groups>
		<Very low + low >	<Inter - +high + very high>
Median (min.–max.)	Total	Mild MDS	Severe MDS	pvalue
Median (min.–max.)	(n = 31)	(n = 13)	(n = 18)	pvalue
Sex (M/F)	19/12	7/6	12/6	0.71
Age at Dx of MDS	50 (27–75)	45 (30–67)	50 (27–75)	0.54
HbG (g/dl)	9.4 (4.8–16.4)	11.4 (5.5–16.4)	9.0 (4.8–13.9)	0.06
ANC (× 10⁹/L)	1.84 (0.01–7.54)	1.46 (0.45–6.97)	2.74 (0.01–7.54)	0.68
PLT (× 10⁹/L)	65 (6–219)	45 (14–219)	69 (6–196)	0.31
WHO subgroup: n (%)
RA/RARS	20 (65)	13 (100)	7 (39)	<0.001
RCMD	5 (16)	0 (0)	5 (28)	0.058
RAEB-1,2	6 (19)	0 (0)	6 (33)	0.02
Karyotype*: n (%)
Good type	2 (7)	2 (15)	0	0.16
Intermediate type	24 (77)	11 (85)	13 (72)	0.66
Poor type	5 (16)	0 (0)	5 (28)	0.058
RBC transfusion dependency**: n (%)	11 (35)	0 (0)	11 (61)	<0.001

(*) Cytogenetics was as follows. Good type: normal, -Y, del(5q), del(20q); poor type: complex (≥ three abnormalities), chromosome 7 anomalies; and intermediate type: other abnormalities.
(**) RBC transfusion dependency was defined as having at least one RBC transfusion every eight weeks over a period of four months. ANC, absolute neutrophil count; Dx, diagnosis; HbG, hemoglobin; MDS, myelodysplastic syndrome; PLT, platelets; RA, refractory anemia; RAEB, refractory anemia with blasts; RARS, refractory anemia with ringed sideroblasts; RBC, red blood cell; RCMD, refractory anemia with multilineage dysplasia; WHO, World Health Organization; WPSS, WHO classification-based prognostic scoring system.

ISSN: 1471-2466