Treatment of Severe Aplastic Anemia with Combined Immunosuppression (Antithymocyte Globulin and High-Dose Methylprednisolone)

Hinterberger-Fischer, M; Hinterberger, W; Höckern, P.; Schmidmeier, W; Gadner, Helmut; Geißler, Klaus; Kos, Mitja; Schwarzinger, I.; Neumann, E; Niessner, H; Schindler, Joanne; Lechner, Klaus

doi:10.1159/000206055

Cited by 17 publications

(3 citation statements)

References 6 publications

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“…Recovery of BM function following immunosuppressive treatment is generally slow, and a totally normal peripheral blood count is obtained only in some of the patients who no longer need transfusions [54,60]. Even when the blood counts return to normal, it is possible to demonstrate abnormalities of BM function using in vitro culture techniques [61].…”

Section: Immunosuppressive Treatmentmentioning

confidence: 99%

Pathophysiology and treatment of aplastic anemia

Geißler

2003

Wien Klin Wochenschr

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Aplastic anemia (AA) is a rare hematological disease characterized by peripheral blood pancytopenia and a hypocellular bone marrow in which normal hematopoietic tissue is replaced by fatty marrow. There is strong in vitro and in vivo evidence suggesting an immunologic mechanism for hematopoietic suppression in the majority of patients with AA. Interferon-gamma and tumor necrosis factor-alpha are considered as soluble mediators of bone marrow (BM) suppression in AA. The events triggering the aberrant immune response are less clear but some viruses and drug metabolites may lead to autoimmune destruction of hematopoietic cells. Patients with severe AA who are younger than 35 to 45 years and who have an HLA-identical sibling donor have a 60-80% chance of being cured by allogeneic BM transplantation. In patients surviving more than two years, chronic graftversus-host disease is the major cause of morbidity and mortality and a solid-tumor malignancy may develop in a few patients. Patients without HLA-identical BM donors and patients older than 35 to 45 years are candidates for combined immunosuppressive treatment with antithymocyte globulin, methylpredisolone and cyclosporine, leading to hematological responses in 70-80% of patients. One has to consider, however, that a significant proportion of these patients will develop further clonal hematological disorders such as paroxysmal nocturnal hemoglobinuria and myelodysplastic syndrome.

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Section: Immunosuppressive Treatmentmentioning

confidence: 99%

Pathophysiology and treatment of aplastic anemia

Geißler

2003

Wien Klin Wochenschr

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“…After failure of GM-CSF therapy (February 1989) and repeated bacterial and viral infections, bleedings and refractoriness to platelet transfusions, ATG was considered in May 1989. Horse ATG (ATGAM, Upjohn, USA) 15 mg/kg/day was given together with 6-methylprednisolone 20 mg/kg/day, as described previously [18]. On the second day, 2 h after completing the second ATG infusion, the patient developed sudden neck stiffness, headache, vomiting and became extremely sensitive to light.…”

Section: Case Reportsmentioning

confidence: 99%

“…In vitro studies have revealed abnormalities of im mune function in SAA [4][5][6][7][8][9], including altered T cell developed thyroid disorders (1 patient with IHT and 1 patient with subacute thyroiditis) several years after suc cessful treatment of SAA with antithymocyte globulin and high-dose methylprednisolone [18].…”

Section: Introductionmentioning

confidence: 99%

Coincidence of Severe Aplastic Anaemia with Multiple Sclerosis or Thyroid Disorders

Hinterberger-Fischer¹,

Kier²,

Forstinger³

et al. 1994

Acta Haematol

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Five patients with severe aplastic anaemia (SAA) who, simultaneously (n = 3) or consecutively (n = 2), presented with multiple sclerosis (MS) (n = 2) or immune hyperthyroidism (IHT) (n = 2) or subacute thyroiditis (n = 1) are described. Two female patients with MS developed SAA after a small dose of azathioprine. Another patient simultaneously presented with IHT and SAA. SAA and MS responded to cyclosporine while IHT required ¹³¹I. Relapsing SAA in 1 patient with MS was treated with antithymocyte globulin (ATG) which induced acute exacerbation of MS. Despite the low total dose of ATG (31.5 mg/kg), complete remission of SAA was obtained. Two other patients developed thyroid disorders, 42 and 106 months after successful immunosuppression with ATG/high-dose methylprednisolone. IHT and subacute thyroiditis were successfully treated with ¹³¹I or prednisolone, respectively, without recurrence of SAA in both cases. These are the first documented cases of SAA evolving in the course of MS while the coincidence with IHT was already described. Since enhanced expression of interferon-γ plays a crucial role in SAA as well as in MS and in IHT, similar pathogenetic principles may apply for these seemingly unrelated disorders.

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Drug-Induced Blood Disorders

Peterson¹

1992

Applied Therapeutics

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Treatment of Severe Aplastic Anemia with Combined Immunosuppression (Antithymocyte Globulin and High-Dose Methylprednisolone)

Cited by 17 publications

References 6 publications

Pathophysiology and treatment of aplastic anemia

Pathophysiology and treatment of aplastic anemia

Coincidence of Severe Aplastic Anaemia with Multiple Sclerosis or Thyroid Disorders

Drug-Induced Blood Disorders

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