Recurrence of Hepatitis-Associated Aplastic Anemia after a 10-year Interval

Muta, Tsuyoshi; Tanaka, Yoshinori; Takeshita, Eiji; Kobayashi, Yuichi; Tokuyama, Takeshi; Ueda, Yôko; Joko, Koji; Fujisaki, Tomoaki; Yokota, Eisuke

doi:10.2169/internalmedicine.47.0933

Cited by 5 publications

(5 citation statements)

References 23 publications

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“…The residing of CD8 cells in bone marrow during HAAA produces a high level of interferon gamma (INF-γ) and cells derived from bone marrow locating in liver may activate these cytotoxic T cells causing their intrahepatic accumulation strongly affected by the Tumor necrosis factor alpha (TNF-α) and interferon gamma (INF-γ) causing the onlooker damage to liver cell of genetically modified mouse model. However, it has also been shown in several studies that increased level of soluble IL-2 receptor forms the major reason of non specific inflammation of HAAA [47,48]. The pathogenesis of HAAA in children has been suggested to relate with the interruption in balance of lymphocyte sub-populations and T lymphocyte activation [49].…”

Section: Immunopathogenesis Of Haaamentioning

confidence: 99%

“…Most of the clinical features relating to aplastic anemia following the hepatitis include: Pallor and multiple skin bleeding [11], lymphocytopenia, hypogammaglobulin [51] low number of CD8/T cell ratio [12] and increased number of cytotoxic cells [48] Neutropenia, fever [17]. Bacterial and fungal infection may emerge as secondary in presenting the disease [2].…”

Section: Clinical Featuresmentioning

confidence: 99%

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Hepatitis Associated Aplastic Anemia: A review

Rauff

Idrees

Shah

et al. 2011

Virol J

124

139

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Hepatitis-associated aplastic anemia (HAAA) is an uncommon but distinct variant of aplastic anemia in which pancytopenia appears two to three months after an acute attack of hepatitis. HAAA occurs most frequently in young male children and is lethal if leave untreated. The etiology of this syndrome is proposed to be attributed to various hepatitis and non hepatitis viruses. Several hepatitis viruses such as HAV, HBV, HCV, HDV, HEV and HGV have been associated with this set of symptoms. Viruses other than the hepatitis viruses such as parvovirus B19, Cytomegalovirus, Epstein bar virus, Transfusion Transmitted virus (TTV) and non-A-E hepatitis virus (unknown viruses) has also been documented to develop the syndrome. Considerable evidences including the clinical features, severe imbalance of the T cell immune system and effective response to immunosuppressive therapy strongly present HAAA as an immune mediated mechanism. However, no association of HAAA has been found with blood transfusions, drugs and toxins. Besides hepatitis and non hepatitis viruses and immunopathogenesis phenomenon as causative agents of the disorder, telomerase mutation, a genetic factor has also been predisposed for the development of aplastic anemia. Diagnosis includes clinical manifestations, blood profiling, viral serological markers testing, immune functioning and bone marrow hypocellularity examination. Patients presenting the features of HAAA have been mostly treated with bone marrow or hematopoietic cell transplantation from HLA matched donor, and if not available then by immunosuppressive therapy. New therapeutic approaches involve the administration of steroids especially the glucocorticoids to augment the immunosuppressive therapy response. Pancytopenia following an episode of acute hepatitis response better to hematopoietic cell transplantation than immunosuppressive therapy.

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Section: Immunopathogenesis Of Haaamentioning

confidence: 99%

Section: Clinical Featuresmentioning

confidence: 99%

Hepatitis Associated Aplastic Anemia: A review

Rauff

Idrees

Shah

et al. 2011

Virol J

124

139

View full text Add to dashboard Cite

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“…The myelotoxic effect in B19 or other viral infections is thought to be due to increased circulating cytotoxic CD8 + T cells and IFN- γ secretion by these cells. Similarly, high circulating CD8 + T cells cause the altered and defective monocyte and macrophage differentiation, decreased level of circulating IL-1, and increased secretion of TNF- α , IFN- γ , and IL-2 receptors which causes onlooker damage of hepatocytes and subsequently occurrence of acute hepatitis [59, 60]. …”

Section: Parvovirus B19 and Hepatitismentioning

confidence: 99%

“…Similarly, high circulating CD8 + T cells cause the altered and defective monocyte and macrophage differentiation, decreased level of circulating IL-1, and increased secretion of TNF- α , IFN- γ , and IL-2 receptors which causes damage of hepatocytes and subsequently leading to acute hepatitis [58–60]. Diagrammatic summary is depicted in Figure 4(b).…”

Section: Pathogenesismentioning

confidence: 99%

Parvovirus B19 Associated Hepatitis

Bihari

Rastogi

Saxena

et al. 2013

Hepatitis Research and Treatment

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Parvovirus B19 infection can present with myriads of clinical diseases and syndromes; liver manifestations and hepatitis are examples of them. Parvovirus B19 hepatitis associated aplastic anemia and its coinfection with other hepatotropic viruses are relatively underrecognized, and there is sufficient evidence in the literature suggesting that B19 infections can cause a spectrum of liver diseases from elevation of transaminases to acute hepatitis to fulminant liver failure and even chronic hepatitis. It can also cause fatal macrophage activation syndrome and fibrosing cholestatic hepatitis. Parvovirus B19 is an erythrovirus that can only be replicate in pronormoblasts and hepatocytes, and other cells which have globosides and glycosphingolipids in their membrane can also be affected by direct virus injury due to nonstructural protein 1 persistence and indirectly by immune mediated injury. The virus infection is suspected in bone marrow aspiration in cases with sudden drop of hemoglobin and onset of transient aplastic anemia in immunosuppressed or immunocompetent patients and is confirmed either by IgM and IgG positive serology, PCR analysis, and in situ hybridization in biopsy specimens or by application of both. There is no specific treatment for parvovirus B19 related liver diseases, but triple therapy regimen may be effective consisting of immunoglobulin, dehydrohydrocortisone, and cyclosporine.

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“…NS1 expression significantly upregulates p21/WAF1 expression, a cyclin-dependent kinase inhibitor that induces G1 arrest leading to apoptosis by activation of caspase-3 and caspase-9 [17, 18]. On the other hand, HPVB19 infection reportedly induces VAHS, which increases circulating CD8 + cytotoxic T cells and IFN- γ and TNF- α secretion, triggering symptoms such as high fever, liver injury, enlarged liver and spleen, coagulation factor abnormalities, pancytopenia, and a build-up of histiocytes in various tissues resulting in the destruction of blood-producing cells [19–21]. In the present case, the bone marrow did not show hemophagocytosis but showed aplastic anemia, indicating that VAHS did not primarily participate in the onset of acute hepatitis.…”

Section: Discussionmentioning

confidence: 99%

Severe Aplastic Anemia following Parvovirus B19-Associated Acute Hepatitis

Furukawa

Kaji

Masuda

et al. 2017

Case Reports in Hepatology

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Human parvovirus (HPV) B19 is linked to a variety of clinical manifestations, such as erythema infectiosum, nonimmune hydrops fetalis, and transient aplastic anemia. Although a few cases have shown HPVB19 infection as a possible causative agent for hepatitis-associated aplastic anemia (HAAA) in immunocompetent patients, most reported cases of HAAA following transient hepatitis did not have delayed remission. Here we report a rare case of severe aplastic anemia following acute hepatitis with prolonged jaundice due to HPVB19 infection in a previously healthy young male. Clinical laboratory examination assessed marked liver injury and jaundice as well as peripheral pancytopenia, and bone marrow biopsy revealed severe hypoplasia and fatty replacement. HPVB19 infection was diagnosed by enzyme immunoassay with high titer of anti-HPVB19 immunoglobulin M antibodies. Immunosuppressive therapy was initiated 2 months after the onset of acute hepatitis when liver injury and jaundice were improved. Cyclosporine provided partial remission after 2 months of medication without bone marrow transplantation. Our case suggests that HPVB19 should be considered as a hepatotropic virus and a cause of acquired aplastic anemia, including HAAA.

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Recurrence of Hepatitis-Associated Aplastic Anemia after a 10-year Interval

Abstract: Hepatitis-associated aplastic anemia (HAA)

Cited by 5 publications

References 23 publications

Hepatitis Associated Aplastic Anemia: A review

Hepatitis Associated Aplastic Anemia: A review

Parvovirus B19 Associated Hepatitis

Severe Aplastic Anemia following Parvovirus B19-Associated Acute Hepatitis

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