Table 2.
Flow Cytometric Findings for 17 Patients With HLH
| Case No. |
Diagnosis | Lympho- cytes (%) |
Lymphocyte Subsets (%) |
Immunophenotype, CD8+ T Cells |
Characteristics of Myeloid Cells | |||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| B | NK | T | CD4/ CD8 |
CD3 Loss |
CD5 Loss |
CD7 Loss |
HLA- DR Gain |
Granulo- cytes (%) |
Abnormal Side Scatter |
CD13 Loss |
CD33 Loss |
Decreased CD10+ Cells |
HLA- DR Gain |
|||
| 1 | EBV | 22 | 2 | 1 | 19 | 0.85 | N | N | Y | ND | 36 | N | ND | N | Y | Y |
| 2 | EBV | 49 | 2 | 3 | 44† | 0.17 | N | N | N | Y | 33 | N | N | N | Y | Y |
| 3 | EBV | 37 | 1 | 1 | 35 | ND | N | N | N | Y‡ | 49 | N | N | N | Y | Y |
| 4 | EBV | 12 | 1 | <1 | 11 | 0.13 | Y | Y | N | Y | 72 | N | ND | N | Y | Y |
| 5 | EBV | 75 | 2 | 16 | 57 | 0.33 | N | Y | Y | Y‡ | 8 | N | ND | N | Y | Y |
| 6 | EBV | 66 | <1 | 7 | 59 | 0.21 | N | Y | Y | Y | 9 | N | N | N | Y | Y |
| 7 | EBV | 16 | 1 | <1 | 15 | 0.08 | N | Y | N | Y | 65 | N | Y | N | Y | Y |
| 8* | EBV/ DLBCL | 3 | <1§ | <1 | 2 | 3.09 | N | N | N | Y | 87 | N | ND | N | Y | Y |
| 9 | EBV/PTCL | 13 | <1 | <1 | 12‖ | 1.35 | N | Y | Y | ND | 54 | N | ND | N | Y | Y |
| 10* | MUNC13 mutation | 91 | 13 | 9 | 69 | 2.53 | N | N | Y¶ | Y | 6 | N | ND | N | N | N |
| 11 | Familial HLH | 24 | 4 | ND | 18 | 7.48 | N | N | N | Y‡ | 51 | N | ND | N | ND | N |
| 12* | Tuberculosis | 5 | <1 | <1 | 5 | 2.7 | N | N | ND | Y | 86 | N | ND | N | Y | Y |
| 13 | B-ALL | 93 | <1 | 82 | 11 | 2.7 | N | N | N | N | 4 | Y | ND | N | ND | Y |
| 14 | Still disease | 61 | 4 | 2 | 55 | 0.52 | N | N | N | Y | 8 | N | ND | N | N | N |
| 15 | JRAf | 41 | 8 | 1 | 41 | 0.31 | N | N | N | Y | 49 | N | ND | N | N | Y |
| 16 | JRA | 11 | 9 | 1 | 1 | 7.5 | N | N | N | Y | 68 | N | N | N | N | N |
| 17 | JRA | 41 | 34 | 0 | 7 | ND | N | N | N | N | 20 | Y | Y | Y | Y | Y |
B-ALL, precursor B acute lymphoblastic leukemia; DLBCL, diffuse large B-cell lymphoma; EBV, Epstein-Barr virus; HLH, hemophagocytic lymphohistiocytosis; JRA, juvenile rheumatoid arthritis; N, no; ND, not done; NK, natural killer; PTCL, peripheral T-cell lymphoma; Y, yes.
*
Peripheral blood was used in cases 8, 10, and 12; in the other cases, bone marrow was used.
†
Rare large granular lymphocytes.
‡
Immunophenotype of CD8+ T cells was not specifically determined, but this immunophenotype was seen on the majority of T cells.
§
Includes neoplastic B cells.
‖
Includes neoplastic T cells.
¶
Also seen on CD4+ T cells.