Table 5.
Presence or absence of HLH-2004 clinical criteria in each patient reviewed.
| Patient | Sex | Age | Comorbidity | Fever | Cytopenia | Splenomegaly | Ferritin | Tiglycerides/fibrinogen | Reduced NK-cell activity | s-IL2 R | Hemophagocytosis | SUM | References |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | M | 18 | 6 | (11) | |||||||||
| 2 | M | 71 | 4 | (12) | |||||||||
| 3 | M | 27 | HIV | 5 | (13) | ||||||||
| 4 | M | 63 | Mb Crohn | 4 | (14) | ||||||||
| 5 | M | 35 | Mb Crohn | 5 | (15) | ||||||||
| 6 | F | 52 | UC | 5 | (16) | ||||||||
| 7 | F | 32 | UC | 4 | (17) | ||||||||
| 8 | M | 22 | Mb Crohn | 3 | (17) | ||||||||
| 9 | F | 32 | Mb Crohn | 6 | (18) | ||||||||
| 10 | F | 28 | Mb Crohn | 4 | (19) | ||||||||
| 11 | F | 33 | Mb Crohn | 4 | (19) | ||||||||
| 12 | F | 30 | Mb Crohn | 3 | (19) | ||||||||
| 13 | F | 38 | Mb Crohn | 4 | (19) | ||||||||
| 14 | M | 36 | Mb Crohn | 4 | (20) | ||||||||
| 15 | M | 21 | HIV | 7 | (21) | ||||||||
| 16 | F | 70 | Sjogren, HT | 6 | (22) | ||||||||
| 17 | M | 72 | HT, HL | 3 | (23) | ||||||||
| 18 | M | 21 | 5 | (24) | |||||||||
| 19 | F | 32 | 6 | (25) | |||||||||
| 20 | F | 48 | IBS | 5 | (26) | ||||||||
| 21 | F | 39 | Obesity, DIA | 8 | (27) | ||||||||
| 22 | X | 30 | HSCT | 2 | (28) | ||||||||
| 23 | X | 18 | HSCT | 2 | (28) | ||||||||
| 24 | X | 56 | HSCT | 2 | (28) | ||||||||
| 25 | F | 50 | KTx | 4 | (29) | ||||||||
| 26 | F | 22 | SLE | 3 | (30) | ||||||||
| 27 | F | 37 | SLE | 6 | (31) | ||||||||
| 28 | M | 35 | UC | 6 | (32) | ||||||||
| 29 | F | 20 | Mb Crohn | 5 | (33) | ||||||||
| 30 | M | 22 | Endocarditis | 3 | (34) | ||||||||
| 31 | F | 44 | SLE | 6 | (35) | ||||||||
| 32 | M | 29 | HIV | 5 | (36) | ||||||||
| 33 | F | 40 | PL, HSCT | 6 | (37) | ||||||||
| 34 | M | 62 | CLL | 5 | (38) | ||||||||
| 35 | F | 52 | AML, HSCT | 2 | (39) | ||||||||
| 36 | F | 27 | 6 | (40) | |||||||||
| 37 | F | 35 | Pregnant | 5 | (41) | ||||||||
| 38 | F | 21 | IM, obesity | 7 | (42) | ||||||||
| 39 | M | 25 | SLE | 4 | (43) | ||||||||
| 40 | M | 63 | KTx, DIA, HT | 3 | (44) | ||||||||
| 41 | F | 24 | Mb Crohn | 4 | (45) | ||||||||
| 42 | M | 40 | KTx | 5 | (46) | ||||||||
| 43 | F | 39 | Pregnant, KTx | 6 | (47) | ||||||||
| 44 | F | 22 | GATA mutation | 7 | (48) | ||||||||
| 45 | F | 31 | MG | 5 | (49) | ||||||||
| 46 | M | 22 | Mb Crohn | 3 | (50) | ||||||||
| 47 | M | 34 | AIDS, BL | 3 | (51) | ||||||||
| 48 | F | 69 | 4 | (52) | |||||||||
| 49 | F | 44 | UC | 5 | (53) | ||||||||
| 50 | F | 59 | UC | 2 | (54) | ||||||||
| 51 | F | 63 | KTx, DIA | 6 | (55) | ||||||||
| 52 | F | 47 | MG | 8 | (56) | ||||||||
| 53 | F | 80 | AOSD | 3 | (57) | ||||||||
| 54 | M | 51 | 6 | (58) | |||||||||
| 55 | M | X | UC, BPD | 5 | (59) | ||||||||
| 56 | F | 18 | ALL | 5 | (60) | ||||||||
| 57 | F | X | DM | 5 | (61) | ||||||||
| 58 | M | 38 | ALL | 4 | (62) | ||||||||
| 59 | F | 49 | LTx | 4 | (63) | ||||||||
| 60 | F | 26 | AOSD | 1 | (64) | ||||||||
| 61 | M | 80 | Brain tumor, TB | 6 | (65) | ||||||||
| 62 | M | 48 | LETM | 5 | (66) | ||||||||
| 63 | M | 50 | KTx | 3 | (67) | ||||||||
| 64 | M | 35 | KTx | 4 | (67) | ||||||||
| 65 | M | 50 | UC, HIV | 5 | (68) | ||||||||
| 66 | F | 51 | Heart failure | 6 | (69) | ||||||||
| 67 | F | 69 | GPA | 4 | (70) | ||||||||
| 68 | F | 63 | Mb Crohn | 6 | (71) | ||||||||
| 69 | M | 23 | Mb Crohn | 4 | (71) | ||||||||
| 70 | F | 62 | ALL | 5 | (72) | ||||||||
| 71 | M | 46 | KTx | 3 | (73) | ||||||||
| 72 | F | 28 | KTx | 3 | (73) | ||||||||
| 73 | F | 72 | 2 | (74) | |||||||||
| 74 | F | 23 | UC | 7 | Present | ||||||||
| SUM | 71 | 57 | 36 | 61 | 32 | 6 | 13 | 62 |
The table illustrates which of the diagnostic criteria are present in each patient. Red cells illustrate that a criterion is confirmed present, green cells mean that a criterion is confirmed not present, and white cells represent data unspecified.
X, Age or gender unknown/unspecified.
HIV, human immunodeficiency virus; UC, ulcerous colitis; HT, hypertension; HL, hyperlipidemia; IBS, irritable bowel syndrome; DIA, diabetes; HSCT, hematopoietic stem cell transplantation; KTx, kidney transplantation; SLE, systemic lupus erythematosus; PL, promyelocytic leukemia; CLL, chronic lymphatic leukemia; AML, acute myeloid leukemia; IM, inflammatory myositis; MG, myasthenia gravis; AIDS, acute immunodeficiency syndrome; BL, Burkitt lymphoma; AOSD, adult-onset Still's disease; BPD, bipolar disorder; DM, dermatomyositis; LTx, liver transplantation; TB, tuberculosis; LETM, longitudinal extensive transverse myelitis; GPA, granulomatosis with polyangiitis.