Steroid-resistant autoimmune myelofibrosis in a patient with autoimmune hepatitis and Evans syndrome complicated with increased expression of TGF-β in the bone marrow: a case report

Ohkawara, Hiroshi; Furukawa, Miki; Ikeda, Kazuhiko; Shichishima-Nakamura, Akiko; Fukatsu, Masahiko; Sano, Tsuyoshi; Ueda, Koki; Kimura, Satoshi; Kanai, Risa; Oka, Yuka; Murakami, Fumi; Сузукі, Осаму; Hashimoto, Yuko; Ogawa, Kaoru; Ikezoe, Takayuki

doi:10.1007/s12185-017-2268-3

Cited by 3 publications

(5 citation statements)

References 24 publications

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“…The relationship between ES and autoimmune hepatitis has been reported by few centers, with main clinical features including pallor, fatigue and splenomegaly. 65 Diagnosis for autoimmune hepatitis and ES is confirmed by the presence of elevated hepatic enzymes, ANA, anti-smooth muscle antibody, DAT, PA-IgG and a confirmatory biopsy with rosette formation. 66 Initial treatment with prednisolone and rituximab is recommended to prevent refractoriness as observed with some immunosuppressants such as azathioprine, mycophenolate mofetil (MMF) and intravenous immunoglobulin (IVIG).…”

Section: Classificationmentioning

confidence: 99%

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Evans syndrome: clinical perspectives, biological insights and treatment modalities

Jaime‐Pérez¹,

Aguilar-Calderón²,

Salazar‐Cavazos³

et al. 2018

JBM

113

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Evans syndrome (ES) is a rare and chronic autoimmune disease characterized by autoimmune hemolytic anemia and immune thrombocytopenic purpura with a positive direct anti-human globulin test. It is classified as primary and secondary, with the frequency in patients with autoimmune hemolytic anemia being 37%–73%. It predominates in children, mainly due to primary immunodeficiencies or autoimmune lymphoproliferative syndrome. ES during pregnancy is associated with high fetal morbidity, including severe hemolysis and intracranial bleeding with neurological sequelae and death. The clinical presentation can include fatigue, pallor, jaundice and mucosal bleeding, with remissions and exacerbations during the person’s lifetime, and acute manifestations as catastrophic bleeding and massive hemolysis. Recent molecular theories explaining the physiopathology of ES include deficiencies of CTLA-4, LRBA, TPP2 and a decreased CD4/CD8 ratio. As in other autoimmune cytopenias, there is no established evidence-based treatment and steroids are the first-line therapy, with intravenous immunoglobulin administered as a life-saving resource in cases of severe immune thrombocytopenic purpura manifestations. Second-line treatment for refractory ES includes rituximab, mofetil mycophenolate, cyclosporine, vincristine, azathioprine, sirolimus and thrombopoietin receptor agonists. In cases unresponsive to immunosuppressive agents, hematopoietic stem cell transplantation has been successful, although it is necessary to consider its potential serious adverse effects. In conclusion, ES is a disease with a heterogeneous course that remains challenging to patients and physicians, with prospective clinical trials needed to explore potential targeted therapy to achieve an improved long-term response or even a cure.

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Section: Classificationmentioning

confidence: 99%

“…Improvement in cytopenias and normalization of hepatic enzymes have been observed, mainly with rituximab, in a year of follow-up. 65 , 67 – 69 …”

Section: Classificationmentioning

confidence: 99%

Evans syndrome: clinical perspectives, biological insights and treatment modalities

Jaime‐Pérez¹,

Aguilar-Calderón²,

Salazar‐Cavazos³

et al. 2018

JBM

113

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Section: Discussionmentioning

confidence: 99%

“…While we have learned much about the bone marrow cytoarchitectural and serological features of AIMF,11 few have examined its genetic or molecular mechanisms. Efforts have been made to characterise serological 'snapshots' of circulating autoantibody and cytokine levels in patients with BMF and AIMF10,19,20 ; others have summarised bone marrow findings in autoimmune conditions more generally,21 even characterizing the T-cell receptor rearrangements of infiltrating T cells. 22 Future work should be systematic, distinguishing between pAIMF, sAIMF, and neither, or between different individual causes of sAIMF (e.g., SLE vs. other), with the aim of establishing pathological/ serological factors correlating with development of BMF and AIMF, as well as with different clinical features.…”

mentioning

confidence: 99%

An overlooked mimic? Autoimmune myelofibrosis—A scoping review of the literature

Riazat-Kesh

Maraveyas

Martin

et al. 2023

European J of Haematology

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Background and ObjectivesAutoimmune myelofibrosis (AIMF) is a rare cause of bone marrow fibrosis (BMF) occurring in the presence or absence of a defined autoimmune disease (secondary or primary AIMF, sAIMF/pAIMF, respectively). Unlike primary myelofibrosis (PMF), AIMF responds well to immunosuppressive therapy with a benign clinical course. Diagnostic criteria for AIMF in opposition to PMF have been lacking, though recent work has helped better characterise molecular and pathological features of AIMF, improving diagnostic precision.MethodsUsing a modern clinical and pathophysiological understanding of AIMF, we apply scoping review methodology and rigorous case‐criteria to retrospectively analyse the case literature. We examine its patient‐population, describing patient‐associated factors, presentation, bone marrow pathology, genetics, treatment and outcomes.ResultsFifty‐five studies were identified, describing 139 AIMF patients. Patients were mostly young females (~4:1 ratio female:male, median age 40.8 years) and typically presented with cytopenias. Splenomegaly was rare. sAIMF was more common than pAIMF (~3:1 ratio), and most cases responded well to immunosuppressive therapy.ConclusionsOur results strengthen the emerging picture of AIMF's patient population, natural history and response to treatment. Further work should continue to use reproducible diagnostic criteria, and explore AIMF's pathophysiology, response to different therapies, and sequelae over larger timescales, as well as differences between pAIMF, sAIMF and PMF.

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“…Several cases of post-LT ES had been reported, most of which were associated with chronic GVHD not seen in our patient. [21][22][23][24][25][26].…”

Section: Discussionmentioning

confidence: 99%

Severe Thrombocytopenia Associated With Helicobacter Pylori Infection after Liver Transplantation: Cases Report and Literature Review

Xu¹

2020

AJSCCR

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Steroid-resistant autoimmune myelofibrosis in a patient with autoimmune hepatitis and Evans syndrome complicated with increased expression of TGF-β in the bone marrow: a case report

Cited by 3 publications

References 24 publications

Evans syndrome: clinical perspectives, biological insights and treatment modalities

Evans syndrome: clinical perspectives, biological insights and treatment modalities

An overlooked mimic? Autoimmune myelofibrosis—A scoping review of the literature

Severe Thrombocytopenia Associated With Helicobacter Pylori Infection after Liver Transplantation: Cases Report and Literature Review

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