Table 1. Designed primers for amplification of the whole coding regions

Designed primer
Forward / Reverse / Gene Bank
Complementary DNA
SH2D1A / 5’ GTT GAC TTG TGC CTG GCT GC 3’ / 5’ ATT TGT AGC TCA CCG AAC TG 3’ / NM 002351
STX11 / 5’ TGA ACT GCA GGT GCC TAT 3’ / 5’ GCA ATT CTT GAA CCT GGC GT 3’ / NM 003764
Mun 218-1310 / 5’ TGC CTC TCC GAA GCG GTG AA 3’ / 5’ GGA CAC AAG CTG CTG CAG GA 3’ / NM 199242
Mun 1112 -2282 / 5’ GAA CGT GGT TCT GAG GCT GC 3’ / 5’ GGA TAG ATC CAC CGC TGA TG 3’
Mun 2087-3309 / 5’ GCG CAT GAG CTC CTC AGA GA 3’ / 5’ GGT GAA GGT CCT TCT TGT GC 3’
Mun 3076-3746 / 5’ GAA TCC AGC AGC AGG CAG AA 3’ / 5’ AAG TGT TAG GCC AGG CTG GA 3’
Genomic DNA
PRF1 Exon 2 / 5’ CCC TTC CAT GTG CCC TGA TAA TC 3’ / 5’ AAG CAG CCT CCA AGT TTG ATT G 3’ / NT 008583
PRF1 Exon 3 / 5’ CCA GTC CTA GTT CTG CCC ACT TAC 3’ / 5’ GAA CCC CTT CAG TCC AAG CAT AC 3’
Table 2. Hematologic laboratory examinations, treatments and prognosis of patients with hemophagocytic lymphohistiocytosis (HLH) at the critical stage #
Patient /Sex / Age
(y) / Onset year
(AD) / Hb
(g/dl) / Absolute count / A-lym
(%) / PLT
(/mm3) / PT
(s) / aPTT
(s) / D-dimmer
(ng/mL) a / Fibrinogen (mg/dL) b / TG
(mg/dL) b / Ferrtin
(ng/ml) b / AST/ALT
(U/L) b / HSP/LAP / Treatment / Deceased after treatment
Lym / Neu
(/mm3)
EBV-associated survival
P7 / M / 6Y2M / 1995 / 11.3 / 1,432 / 2,452 / 0 / 240,000 / 17 / 35 / NA / NA / 332 / NA / 326 / 118 / +/+ / IVIG/ST/VP16
P9 / F / 3Y6M / 1998 / 11 / 3,896 / 3,587 / 36 / 12,000 / 22 / 50 / NA / NA / NA / NA / 762 / 170 / +/- / IVIG/ST
P15 / M / 10Y7M / 2001 / 7.8 / 867 / 1,770 / 1 / 35,000 / 13 / 26 / NA / NA / NA / NA / 16 / 10 / +/+ / ST
P21 / M / 1Y / 2005 / 8.2 / 2,135 / 2,806 / 7 / 20,000 / 17 / 63 / 701 / 173 / 239 / 13,906 / 603 / 358 / +/- / IVIG/ST/CsA/VP16
P28 /F / 2Y10M / 2006 / 8.3 / 4,290 / 990 / 3 / 90,000 / 16 / 37 / NA / NA / 177 / 751 / 2,127 / 1,665 / +/- / IVIG/ST/CsA
P29 / F / 6Y6M / 2006 / 8.5 / 3,192 / 8,246 / 7 / 56,000 / 14 / NA / 2,711 / 158 / 255 / 2,404 / 342 / 214 / +/- / IVIG/ST/CsA/VP16
EBV-associated dead
P2 / M / 3Y2M / 1992 / 7.0 / 1,300 / 1,850 / 37 / 26,000 / 19 / 74 / 3-4 / NA / NA / NA / 471 / 204 / +/+ / IVIG/ST / 14 days
P4 / M / 6Y6M / 1993 / 5.5 / 1,205 / 384 / 2 / 93,000 / 100 / 100 / 500-1000 / 80 / 234 / NA / 2,898 / 1,285 / +/+ / IVIG / 8 dyas
P14 / M / 1Y10M / 2001 / 7.9 / 290 / 540 / 0 / 14,000 / 11 / 45 / 5,673 / NA / 295 / 14,523 / 358 / 253 / +/- / IVIG/ST/G-CSF / 29 days
P18 / F / 1Y7M / 2002 / 5.1 / 446 / 36 / 4 / 7,000 / 100 / 100 / NA / 47 / 473 / 15,437 / 2,109 / 485 / +/+ / IVIG / 13 days
P23 / F / 5Y2M / 2005 / 8.4 / 4,120 / 1,204 / 23 / 49,000 / 12 / 34 / NA / 264 / 506 / 334 / 63 / 23 / +/+ / IVIG/ST / 58 days
P25 /M / 11Y / 2005 / 8.3 / 340 / 60 / 4 / 67,000 / 35 / 48 / NA / 89 / 113 / 345 / 66 / 45 / +/- / IVIG/CsA / 15 days
Non-EBV-associated survival
P3 / F / 12Y3M / 1992 / 11.2 / 1,154 / 1,235 / 8 / 75,000 / NA / NA / NA / NA / 243 / 2,785 / 237 / 174 / +/+ / ST
P10 / M / 4M / 1998 / 7.9 / 2,451 / 7,800 / 0 / 18,200 / 37 / 42 / NA / 134 / 165 / 3,479 / 42 / 34 / +/- / IVIG
P13 / F / 1Y5M / 1999 / 4.9 / 784 / 504 / 0 / 8,000 / 21 / 58 / 485 / 254 / NA / NA / 89/ 154 / +/+ / IVIG
P17 / F / 1Y4M / 2001 / 4.8 / 674 / 925 / 1 / 57,000 / 14 / 30 / NA / 144 / 468 / NA / 843 / 198 / +/- / IVIG
P20 / F / 7M / 2004 / 9.8 / 4,984 / 336 / 5 / 42,000 / 18 / 39 / NA / 124 / 512 / 638 / 1,113 / 1,233 / +/- / None★
P22 / F / 7Y / 2005 / 7.3 / 1,530 / 2,475 / 19 / 37,000 / 16 / 40 / 5,715 / 225 / 300 / 7,572 / 5,425 / 2,163 / +/+ / IVIG/ST/CsA/VP16
P26 /M / 1Y6M / 2006 / 7.9 / 720 / 432 / 4 / 26,000 / 14 / 45 / 7,541 / 198 / 244 / 3,016 / 209 / 48 / +/- / IVIG/ST/VP16
P30/ M / 8Y5M / 2006 / 9.1 / 3,100 / 4,092 / 7 / 24,000 / 13 / 35 / 9,790 / 111 / 483 / 121,560 / 327 / 189 / +/- / IVIG/CsA
Non-EBV-associated dead
P1 / M / 2Y11M / 1992 / 8.2 / 710 / 3,245 / 0 / 77,000 / 18 / 61 / NA / 30 / NA / NA / 875 / 299 / +/- / IVIG / 6 days
P5 / M / 8Y3M / 1994 / 8.5 / 458 / 602 / 0 / 31,000 / 16 / 48 / 1000-2000 / 110 / 569 / NA / 1,565 / 1,069 / +/+ / IVIG/acyclovir / 8 days
P6 / F / 1Y1M / 1995 / 7.2 / 587 / 972 / 0 / 24,000 / 100 / 100 / 1000-2000 / 83 / 313 / 15,438 / 768 / 653 / +/- / IVIG/ST/VP16 / 3 days
Table 2. Hematologic laboratory examinations of patients with hemophagocytic lymphohistiocytosis (HLH) at the critical stage (continued.) #
Patient /Sex / Age
(y) / Onset year
(AD) / Hb
(g/dl) / Absolute count / A-lym (%) / PLT
(/mm3) / PT
(s) / aPTT
(s) / D-dimmer
(ng/mL) a / Fibrinogen
(mg/dL) b / TG
(mg/dL) b / Ferrtin
(ng/mL) b / AST/ALT
(U/L) b / HSP/
LAP / Treatment / Deceased after treatment
Lym / Neu
Non-EBV-associated dead
P8 / F / 2Y2M / 1996 / 6.5 / 1,080 / 90 / 0 / 12,000 / 14 / 39 / 1000-2000 / 54 / 587 / 2,684 / 11 / 43 / +/+ / IVIG/ST/VP16 / 14 days
P11 / F / 6Y5M / 1998 / 6.9 / 1,452 / 3,150 / 0 / 89,000 / 26 / 41 / 3000-4000 / 108 / 643 / 5,743 / 425 / 564 / +/+ / IVIG/ST/VP16 / 27 days
P12 / F / 15Y4M / 1998 / 6.6 / 620 / 440 / 0 / 70,000 / 21 / 70 / 3000-4000 / 75 / 287 / 6,432 / 37 / 54 / +/+ / IVIG/ST / 7 days
P16 / M / 2M / 2001 / 5.2 / 960 / 140 / 6 / 4,000 / 100 / 100 / 4,890 / 70 / 491 / 2,987 / 146 / 75 / +/+ / IVIG/ST/VP16 / 27 days
P19 / M / 15Y6M / 2004 / 12.4 / 437 / 1,587 / 1 / 213,000 / 24 / 67 / 3,476 / 284 / 139 / 2,135 / 409 / 259 / +/+ / IVIG/ST / 61 days
P24 /M / 3M / 2005 / 8.4 / 1,924 / 312 / 19 / 33,000 / 16 / 35 / NA / 156 / 206 / 4,66.9 / 84 / 59 / +/- / IVIG/ST/CsA/VP16 / 8 months
P27 /F / 2M / 2006 / 8.6 / 17,420 / 5,180 / 0 / 154,000 / 64 / 100 / 8,954 / 78 / 367 / 18,945 / 763 / 459 / +/+ / IVIG/ST / 54 days
P31 / M / 4Y8M / 2007 / 8.4 / 650 / 1,500 / 11 / 118,000 / 22.4 / 100 / NA / 134 / 389 / 5,498 / 882 / 321 / +/+ / IVIG / 32 days
P32 / F / 13Y2M / 2007 / 7.7 / 2,300 / 2,567 / 45 / 275,000 / 14.3 / 63.9 / 5,429 / 93 / 653 / 562 / 1,165 / 195 / +/+ / IVIG/ST/CsA/VP16 / 63 days

Abbreviation: M, male; F, female; Dx, diagnosis; Lym, lymphocyte; Neu, neutrophil; A-lym, atypical lymphocyte; PT, prothrombin time; aPTT, activated partial thrombolastin time; TG, triglyceride; AST, aspartate transferase; ALT, alanine transferase; HSP, hepatosplenomegaly; LAP, lymphadenopathy; IVIG, intravenous immunoglobulin; ST, steroid prednisolone or dexamethason; CsA, cyclosporine A; VP-16, etoposide; D, dead; A, alive; NA, not available

# All patients had not insult on central nervous system

a The D-dimmer was evaluated by semi-quantity (presented as a range) or quantity system (presented as a number), their normal range was ＜ 250 ng/ml or ＜ 500ng/ml (F.E.U), respectively. b The normal range of fibrinogen, TG, ferrtin, AST and ALT were 190-380 mg/dL, ＜ 150 mg/ml, 10-322 ng/ml, 13-40 U/L and ＜ 36 U/L, respectively

★HLH spontaneously resolved.