Korean J Hematol.  2007 Mar;42(1):62-66. 10.5045/kjh.2007.42.1.62.

A Case of Hemophagocytic Lymphohistiocytosis Accompanied by Acute Hepatitis A: Review of the Literature

• Lee HJ
• Chung JS
• Shin HJ
• Choi YJ
• Park YE
• Kim PJ
• Kim ID
• Lee EY
• Cho GJ

• KMID: 2252271
• DOI: http://doi.org/10.5045/kjh.2007.42.1.62

Abstract

A previously healthy 26-year-old female was referred to our hospital because of fever and abnormalities of her blood biochemistry. Her laboratory results displayed leukopenia (1.79x103/microliter), thrombocytopenia (85x103/microliter), the serum aspartate aminotransferase and alanine aminotransferase levels were elevated to more than 2,000 IU/L, and the serum levels of lactate dehydrogenase and ferritin were markedly increased. Mild hepatosplenomegaly was reported on the abdomen-pelvis computed tomography. The bone marrow smears revealed proliferation of mature histiocytes that were ingesting platelets and erythrocytes, which is consistent with hemophagocytic lymphohistiocytosis. Although the other viral markers were all negative, the anti-hepatitis A IgM was positive and the anti-hepatitis A IgG was negative. Therefore, the patient was diagnosed as hepatitis A-associated hemophagocytic lymphohistiocytosis. Since a fulminant clinical course was suspected, 2 cycles of cyclosporine (3mg/kg iv from day 1 to day 5), dexamethasone (30mg iv qd from day1 to day 4) and immunoglobulin (500mg/kg iv day 1) therapy was started from the seventh day after onset and a favorable clinical outcome resulted.

Keyword

Hemophagocytic lymphohistiocytosis; Acute hepatitis A; Thrombocytopenia

MeSH Terms

Adult
Alanine Transaminase
Aspartate Aminotransferases
Biochemistry
Biomarkers
Bone Marrow
Cyclosporine
Dexamethasone
Erythrocytes
Female
Ferritins
Fever
Hepatitis A*
Hepatitis*
Histiocytes
Humans
Immunoglobulin G
Immunoglobulin M
Immunoglobulins
L-Lactate Dehydrogenase
Leukopenia
Lymphohistiocytosis, Hemophagocytic*
Thrombocytopenia
Alanine Transaminase
Aspartate Aminotransferases
Cyclosporine
Dexamethasone
Ferritins
Immunoglobulin G
Immunoglobulin M
Immunoglobulins
L-Lactate Dehydrogenase

Figure

• 

  Fig. 1 Abdomen-pelvis computed tomography shows splenomegaly, mild hepatomegaly and periportal low density in liver. Collapsed gallbladder with wall thickening and fluid collection in pelvic cavity are also detected.

• 

  Fig. 2 Bone marrow aspiration showing hemophagocytosis of megakaryocyte (A), and red blood cells (B) (Wright-Giemsa stain, ×400).

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