Peripheral blood mononuclear cell (PBMC) marker analysis in the differential diagnosis of hemophagocytic syndrome (HPS) (Meeting abstract).

Important note: Information in this article was accurate in 1994. The state of the art may have changed since the publication date.

Peripheral blood mononuclear cell (PBMC) marker analysis in the differential diagnosis of hemophagocytic syndrome (HPS) (Meeting abstract).

Proc Annu Meet Am Soc Clin Oncol; 12:A1314 1993. Unique Identifier : AIDSLINE ICDB/94695713
Imashuku S; Hibi S; Kataoka Y; Ikushima S; Fujiwara F; Todo S; Fujita N; Kyoto Prefectural Univ. of Medicine, Kyoto, Japan

Abstract: HPS includes heterogeneous disorders: a virus- or infection-associated HPS(VAHS/IAHS), which must be supportively treated; a familial type HPS(FEL); and a tumor-associated HPS often seen with malignant lymphomas or true histiocytic malignancy, which needs intensive chemotherapy. Since the clinical signs and symptoms caused by hypercytokinemia are quite similar in all cases of HPS, choosing the most appropriate measures for treatment at diagnosis is usually difficult. We studied PBMC markers by two-color analysis in 27 HPS patients (pts) before treatment (median age 1 yr, ranging from 1 mo old to 62 yr old, female n=15) during the-acute phase of the disease. Based on the results, the pts were subclassified into 5 groups (G): G-I (n=7), with over 25% CD3+HLADR+ cells and a low CD4/CD8 ratio, showed a poor outcome unresponsive to therapy; G-II (n=6) with higher than 30% CD19+ cells were all alive, suggesting a prognostically favorable IAHS; G-III (n=6), with a CD4/CD8 ratio higher than 3.0; G-IV (n=2) with a very high percentage of CD56 suggesting natural killer (NK) cell leukemia; and G-V (n=6), with normal subsets. The prognosis of NK cell leukemia/lymphoma (1 in G-I, 1 in G-III and 2 in G-IV) was poor. The outcomes of the pts in G-III and G-V were variable but better, including 2 FEL cases which received allogeneic BMT. These results clearly indicate that PBMC marker studies before treatment are useful in subclassifying HPS and predicting the prognosis and for determination of the most appropriate treatment for each HPS pt.

Keywords: Adolescence Adult Antigens, CD/BLOOD Biological Markers/BLOOD Child Child, Preschool CD4-CD8 Ratio Diagnosis, Differential Female Histiocytosis, Non-Langerhans-Cell/BLOOD/CLASSIFICATION/ *DIAGNOSIS/THERAPY Human Infant Killer Cells, Natural/PATHOLOGY Lymphocyte Subsets/PATHOLOGY Male Middle Age Monocytes/*PATHOLOGY Prognosis ABSTRACT
940228
M9420822

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