Table 2.
Prognostic findings of HLH
| Prognostic indicator | Favorable factor | Adverse factor | Reference |
|---|---|---|---|
| General | |||
| Age | >2 y, <60 y | <2 y, >60 y | 81,82,99-102 |
| Sex | Female | Male | 82 |
| Laboratory values | |||
| Ferritin | <2 000 μg/L | >50 000 μg/L | 83,101,103 |
| Bilirubin level | <2 mg/mL | >2 mg/mL | 78,99,104 |
| Lactate level | <2 000 U/L | >2 000 U/L | 99 |
| Albumin | >20 g/L | <20 g/L | 104 |
| β-2 microglobulin | <4.03 mg/L | >4.03 mg/L | 105 |
| Neutrophils | >500/μL | <500/μL | 104 |
| CD3+ cells | Increased | Decreased | 106 |
| CD8+ cells | >45% of CD3+ cells | <45% of CD3+ cells | 106 |
| CD4:CD8 ratio | Decreased | Maintained or Increased | 106,107 |
| NK cell level | <3% of lymphocytes | >3% of lymphocytes | 107,108 |
| Platelet count | >80 000/μL | <80 000/μL | 100,101 |
| Platelet count normalization | <2 wk | >2 wk | 104 |
| aPTT | <60 s | >60 s | 102 |
| Cholinesterase | >2 000 U/L | <2 000 U/L | 108 |
| IL-10 | >2 000 pg/mL | <2 000 pg/mL | 109 |
| C16 ceramide:sphingosine | Low | High | 110 |
| AST or ALT | <800 IU/L | >800 IU/L | 102 |
| DIC score | <5 | >5 | 101 |
| Physical examination | |||
| Edema | Absent | Present | 78 |
| Splenomegaly | Absent | Present | 82 |
| Spleen size | <4 cm | >4 cm | 111 |
| Disease | |||
| Malignancy | Absent | Present | 83 |
| Lymphoma | Absent | Present | 81 |
| Cancer type | B-cell NHL | T/NK-cell lineage | 78,84 |
| CNS involvement | No involvement | Involvement | 100 |
| Clinical parameters | PET parameters of marrow | PET negative | 112 |
| Treatment | 112 | ||
| Initial therapy | HLH94/04 | Not HLH94/04 | 112 |
| Clinical response at 2 wk | Yes | No | 101 |
| Treatment | Etoposide | No etoposide | 81,100 |
| HSCT stem cell source | BM, PB | Cord | 113 |
| HSCT donor type | MRD, MUD | MMUD | 113 |
Prognostic outcomes specific to malignancy-induced HLH are italicized. Many prognostic implications have been drawn from studies of HLH, but relatively few are isolated to adult HLH, with even fewer being related to malignancy-induced HLH. General features of HLH that are known to yield an adverse prognostic impact are age, ferritin level, bilirubin level, lactate, albumin, β2-microglobulin, neutropenias, thrombocytopenia, and an elevated aPTT. Less conventional laboratory values including elevated IL-10, ratio CD4:CD8, NK-cell levels, cholinesterase, and C16 ceramide:sphingosine ratio are also able to predict poor outcome.
aPTT, activated partial thromboplastin time; ALT, alanine aminotransferase; AST, aspartate aminotransferase; DIC, disseminated intravascular coagulation; MMUD, mismatched unrelated donor; MRD, matched related donor; MUD, matched unrelated donor.