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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 (1.79×103/μL), thrombocytopenia (85×103/μL), 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.
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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).
Table 1.
Laboratory data
Abbreviations: WBC, white blood cell; Hb, hemoglobin; PLT, platelet; PT, prothrombin time; aPTT, activated partial thromboplatin time; AST, aspartate aminotransferase; ALT, alanine aminotranferase; LDH, lactate dehydrogenase; ALP, alkaline phosphatase; GTP, glutamyl transpeptidase; TG, triglyceride; HDL, high-density lipoprotein; CRP, C-reactive protein; HCV, hepatitis C virus; HAV, hepatitis A virus; EBV, Ebstein-barr virus; HSV, herpes simplex virus; CMV, cytomegalovirus; HIV, human immunodeficiency virus.
Table 2.
Case reports of hepatitis A virus associated hemophagocytic lymphohistiocytosis
| Case | Year | Age/Sex | Treatment | sIL-2R (U/mL) | Outcome | Underlying diseas |
|---|---|---|---|---|---|---|
| Watanabe et al (9) | 1988 | 24/F | None | Unknown | Recovered | |
| McPeake et al (10) | 1993 | 20/F | Steroid pulse | Unknown | Recovered | Still's disease |
| Kondo and Date et al (11) | 1995 | 49/F | Steroid pulse, rhG-CSF | 2,080 | Recovered | |
| Wu et al (12) | 1995 | 23/M | Steroid pulse | Unknown | Died | HCV carrier |
| Kyoda et al (13) | 1998 | 40/M | None | Unknown | Recovered | |
| Watanabe et al (9) | 2000 | 36/M | HDF, steroid pulse, combination chemotherapy∗ | Unknown | Died | |
| Onaga et al (8) | 2000 | 19/F | Steoid pulse | 1,920 | Recovered | |
| Watanabe et al (9) | 2002 | 45/M | None | 4,870 | Recovered | |
| 2002 | 41/M | None | 2,590 | Recovered | HCV carrier | |
| Present patient | 2005 | 26/F | Combination chemotherapy† | Unknown | Recovered |
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