Table 1.
Myelodysplastic syndrome cases associated with hemophagocytic Lymphohistiocytosis (HLH)
| Age (years) | Fever | ANC (/mL) | Hb (g/dL) | Platelet (K/UL) | Ferritin (ng/dL) | Fibrinogen (mg/dL) | TAG (mg/dL) | IL-2 (pg/dL) | Hemophagocytosis | Marrow blast (%) | Karyotype | R-IPSS | Hepatomegaly/splenomegaly | HLH-2004 (points) | Outcome | |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | 8 | Yes | 400 | 10.6 | 49 | 1070 | 256 | 306 | 4250 | Yes | 2.2 | 46, XX, + 8 [20] | Inter-mediate | Yes/yes | 5 | Alive |
| 2 | 60 | Yes | 6800 | 6.5 | 14 | 649 | NA | NA | 4054 | Yes | 2 | Complex [including + 8]a | Very high | Yes/yes | 5 | Alive |
| 3 | 68 | NA | NA | 10.5 | 45 | 24,316 | 129 | NA | 1025 | Yes | 0 | 46, XY | Low | No/yes | 5 | Died |
| Case | 83 | Yes | 8900 | 8.9 | 13 | 9479 | 70 | 321 | 34 | Yes | 0 | 47, XX, + 8 [6], 46, XX [5] | Low | No/no | 6 | Died |
ANC absolute neutrophil count, Hb hemoglobin, TAG triglycerides, IL2 Interleukin 2 soluble receptor, IPSS International Prognostic Score System, NA not available
aPatient karyotype = 54-57,XY + 1 [2], + 3 [2], + [4], + 8 [4].add (9) (p22) [2], + 11[3].add (15) (p.11.2) [4], add (16) (q24) [4], add (19) (p13.1) [4], add (20) (p13) [4],?21 [2],2–5mar[cp4]/46,XY [8]