Chronic myelomonocytic leukemia-associated pulmonary alveolar proteinosis: A case report and review of literature

Table 1 Clinical characteristics of published cases of secondary pulmonary alveolar proteinosis associated with myelodysplastic syndrome in adults

Order	Diagnosis	Case number	Age/Gender	Duration from MDS to PAP	Treatment	Anti-GM-CSF Ab	Concomitant disease	Chromosomal abnormality	Genetic abnormality	Survival time after PAP	Outcome	Survival time after MDS	Ref.
1	MDS	1	39/M	1 yr after MDS	NA	NA	Pulmonary fungus	NA	NA	Shortly	Died	1 yr	[21]
2	MDS	1	65/F	Coincidence	Methylprednisolone and GM-CSF	NA	None	NA	NA	Shortly	Died	Shortly	[21]
3	RA deteriorated into AL after 2 yr	1	27/M	1 yr after AL	Antibiotics and anti-tuberculosis agents	NA	None	NA	NA	4 mo	Died	3 yr	[10]
4	RA deteriorated into AL after 1 yr	1	65/F	1 mo after AL	Fluconazole and miconazole	NA	Pulmonary fungus	NA	NA	1 mo	Died	1 yr	[10]
5	RAEB-T	1	52/F	2 yr after RAEB-T	Amphotericin B	NA	Pulmonary fungus	NA	NA	4 mo	Died	1 yr	[10]
6	RA deteriorated into AL within 1 yr	1	70/M	1 yr after AL	Chemotherapy	NA	Malignant melanoma	NA	NA	4 mo	Died	2 yr	[10]
7	RA deteriorated into RAEB after 12 yr	1	50/M	Coincidence with RAEB	Prednisolone	Negative	Hypereosinophil	46, XY, -1,-14,+2mar[1] and 46, XY[3] at first diagnosis and 47, XY, add (1)(p11), +add(1), t(1;19)(q11;q11), -14, +mar at disease progression	NA	2 yr	Alive	14 yr	[22]
8	MDS	1	66/M	12.5 yr	Prednisolone and amobroxol	Negative	NA	47, XY, add(1)(p11), +add(1),t(1;19)(p10; q10), –14, +mar1[20/20]	NA	NA	NA	NA	[23]
9	MDS	1	47/M	2 yr	NA	Negative	NA	NA	NA	Shortly	Died	2 yr	[24]
10	MDS-RA deteriorated into RAEB after 9 yr	1	36/M	1 yr after RAEB	Cord blood transplantation and GM-CSF	Negative	None	NA	NA	1 yr	Alive	11 yr	[25]
11	MDS	1	39/F	NA	BAL	NA	NA	NA	NA	NA	Alive	NA	[8]
12	MDS-RA deteriorated into RAEB after 1 yr	1	48/M	1 yr with RAEB	WLL	Negative	None	47, XY, +8	NA	2 r	Alive	3 yr	[26]
13	RCMD	1	41/F	3 yr	Prednisolone and antibiotics	NA	None	46, XX, –20; der (20)del (20)(q11q13) idic(20) (p11), or idic(20q–)	NA	7 mo	Died	4 yr	[4]
14	MDS-RA	1	34/M	1 yr	Unrelated bone marrow transplantation	Negative	IBD	47, XY, +8	NA	1 yr	Alive	3 yr	[27]
15	MDS-RAEB	1	42/F	4 mo before RAEB	Unrelated cord blood transplantation.	Negative	IBD	47, XX, +8	NA	4 mo	Died	Shortly	[27]
16	MDS-RAEB	1	52/F	8 yr before RAEB	Unrelated bone marrow transplantation	Negative	BD and IBD	47, XX, +8	NA	10 yr	Alive	2 yr	[27]
17	MDS	1	34/M	2 yr before MDS	BAL	NA	NA	NA	NA	NA	NA	NA	[17]
18	MDS-MLD	1	38/M	NA	NA	Negative	NA	NA	NA	NA	Died	< 2 yr	[15]
19	MDS-SLD	1	26/M	NA	NA	Negative	NA	NA	NA	NA	Died	< 2 yr	[15]
20	MDS-U	1	37/M	NA	NA	Negative	NA	NA	NA	NA	Died	< 2 yr	[15]
21	MDS-EB	1	33/M	NA	NA	Negative	NA	NA	NA	NA	Died	< 2 yr	[15]
22	Very low + low MDS	13	Age: 45 (30-67); Gender: 7 M/6 F	MDS to sPAP: 0 to 168 mo 2 case before	NA	Negative	NA	Good: 2; Intermediate: 11	NA	13 mo	7 Died; 6 Alive	NA	[5]
34	Inter-high + very high	18	Age: 50 (27-57); Gender: 12 M/6 F	MDS to sPAP: 0 to 228 mo 6 cases before	NA	Negative	NA	Intermediate: 13; Poor: 5	NA	15 mo	10 Died; 8 Alive	NA	[5]
51	MDS-U deteriorated into RCMD after 11 mo and deteriorated into RAEB-1 after 13 mo	1	75/F	Coincidence with RAEB1	Prednisolone	Negative	Organizing pneumonia	Normal karyotype	NA	6 mo	Died	19 mo	[16]
52	MDS-RA	1	46/F	3 yr	Steroid pulse, cyclosporine A and infliximab	NA	BD and myelofibrosis	Trisomy 8	NA	30 mo	Died	66 mo	[28]
53	MDS-RA	1	31/M	2 yr	Steroid	NA	BD	Trisomy 8	NA	2 mo	Died	2 yr	[28]
54	MDS-RAEB2	1	50/F	Coincidence with RAEB	WLL	NA	BD	Trisomy 8	NA	2 yr	Alive	2 yr	[28]
55	MDS	1	40/M	NA	Stem cell transplantation	NA	NA	NA	NA	NA	Alive	NA	[19]
56	MDS	1	51/F	NA	NA	Negative	LGL, metastatic melanoma, DVT	Normal karyotype	GATA2	NA	Died	12 yr	[29]
57	MDS/AML	1	33/M	Less than 1 yr	NA	Negative	None	-7	GATA2	< 1 yr	Died	< 1 yr	[29]
58	MDS	1	26/F	Less than 1 yr	NA	Negative	Hypothyroidism	Normal karyotype	GATA2	< 1 yr	Died	< 1 yr	[29]
59	MDS	1	26/F	NA	NA	Negative	LGL, breast cancer, miscarriage	Normal karyotype	GATA2	NA	Died	27 yr	[29]
60	MDS/AML	1	19/F	NA	NA	Negative	LGL/DVT	Normal karyotype	GATA2	NA	Died	25 yr	[29]
61	MDS	1	28/M	NA	NA	Negative	None	Trisomy 8	GATA2	NA	Alive	6 yr	[29]
62	MDS	1	18/F	NA	NA	Negative	LGL, miscarriage	Normal karyotype	GATA2	NA	Died	31 yr	[29]
63	CMML	1	48/F	NA	NA	Negative	LGL, HT, Pancreatic cancer	Normal karyotype	GATA2	NA	Died	11 yr	[29]
64	MDS	1	59/F	Very soon	NA	Negative	NA	NA	GATA2	Shortly	Died	Shortly	[30]
65	CMML	1	63/M	Coincidence with CMML-1	Chemotherapy	NA	None	inv(6)	JAK2-V617F	5 yr	Died	5 yr	Current study

AL: Acute leukemia; AML: Acute myelogenous leukemia; BAL: Bronchoalveolar lavage; BD: Behçet’s disease; CMML: Chronic myelomonocytic leukemia; DVT: Deep venous thrombosis; EB: Excess blasts; GM-CSF: Granulocyte-macrophage colony stimulation factor; HT: Hereditary thrombocythemia; IBD: Inflammatory bowel disease; LGL: Large granular lymphocyte; MDS: Myelodysplastic syndrome; MLD: Multilineage dysplasia; NA: Not available; PAP: Pulmonary alveolar proteinosis; RA: Refractory anemia; RAEB: Refractory anemia with excess blasts; RAEB-T: Refractory anemia with excess blasts in transformation; RCMD: Refractory cytopenia with multilineage dysplasia; SLD: Single lineage dysplasia; sPAP: Secondary pulmonary alveolar proteinosis; U: Unclassifiable; WLL: Whole lung lavage.

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