Analysis of triglyceride value in the diagnosis and treatment response of secondary hemophagocytic syndrome.
OBJECTIVE
Secondary hemophagocytic syndrome (hemophagocytic lymphohistiocytosis, HLH) follows viral infection, malignant disorders, and autoimmune disease. Criteria for HLH diagnosis, which were proposed in 2004, include hypertriglyceridemia. However, some studies reported the absence of hypertriglyceridemia in patients with secondary HLH, differing from those with primary HLH.


METHODS
In this study, we investigated the presence or absence of hypertriglyceridemia in 28 patients who were diagnosed with secondary HLH between 1997 and 2007 retrospectively. There were no patients undergoing treatment for those with a history of hyperlipidemia.


RESULTS
The subjects consisted of 14 patients with lymphoma-associated HLH, 11 with virus-associated HLH, 2 with autoimmune disease-associated HLH, and 1 with post transplantation HLH. In 19 patients (68%), hypertriglyceridemia was noted on diagnosis or during the disease period (mean: 242 mg/dL). Furthermore, the triglyceride (TG) level decreased with the treatment-related amelioration of HLH (mean level before and after treatment: 297 and 136 mg/dL, respectively, p=0.0001).


CONCLUSIONS
These results suggest that the TG level is useful for diagnosing HLH and evaluating the treatment response. TG measurement is simple and inexpensive; therefore, this parameter can be determined several times to evaluate the treatment response.
Okamoto
Muneo
M
Division of Hematology, Department of Internal Medicine, Nippon Medical School, Tokyo.
Yamaguchi
Hiroki
H
Isobe
Yasushi
Y
Yokose
Norio
N
Mizuki
Taro
T
Tajika
Kenji
K
Gomi
Seiji
S
Hamaguchi
Hiroyuki
H
Inokuchi
Koiti
K
Oshimi
Kazuo
K
Dan
Kazuo
K
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001327:Autoimmune Diseases / Q000150:complications; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation / Q000009:adverse effects; D006801:Humans; D015228:Hypertriglyceridemia / Q000097:blood / Q000150:complications; D051359:Lymphohistiocytosis, Hemophagocytic / Q000097:blood* / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology; D008223:Lymphoma / Q000150:complications; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D016896:Treatment Outcome; D014280:Triglycerides / Q000097:blood*; D014777:Virus Diseases / Q000150:complications; D055815:Young Adult
D014280:Triglycerides