Objective    To study the clinicopathological characteristics

diagnosis and prognosis of anaplastic large cell lymphoma

lymphohistiocytic variant (ALCL-LH).     Methods    Two cases of ALCL-LH were diagnosed according to the WHO standard of lymphoma classification. Their clinical manifestations were analyzed. Lymphoma sample sections were prepared for pathological features and immunohistochemical assays

and examined by in situ hybridization for Epstein-Barr virus (EBV) DNA. Concerning literatures were reviewed.     Results    Both 2 cases occurred in children. The clinical manifestations presented with fever and enlarged lymph nodes as well as hepatomegaly and splenomegaly. Microscopically

the neoplastic cells

with irregular nuclei

were more often medium to large  in size but smaller than those in the common type

and sparsely intermixed with a large number of histiocytes and lymphocytes that exceeded and masked the tumor cell population. The tumor cells clustered around blood vessels. Case 1 showed evidence of erythrophagocytosis. Immunohistochemical stainings showed that tumor cells of 2 cases were positive to CD30

anaplastic lymphoma kinase (ALK)

EMA

and Granzyme B

as well as case 1 to CD43

and case 2 to CD7. But both cases were negative to CD45

CD2

CD3

CD4

CD5

CD8

CD45RO

CD56

CD20

CD79a

CD15

MPO

CK

TdT

and LMP1. In addition

the EBV DNA was not detected in the lymphoma. Case 1 and case 2 died 10 d and 3 months after pathological diagnosis respectively.     Conclusion    ALCL-LH variant is rare subtype

and often occurs in pediatric patients. Morphologically

ALCL-LH is characterized by a few medium to large size cells admixed with a large number of histiocytes and lymphocytes that exceed and mask the tumor cell population. The expressions of CD30

ALK

EMA and cytotoxic granule associated proteins contribute to diagnosis and differential diagnosis.