Clinical stable systemic mastocytosis with interferon alpha 2b therapy.

Hübner, Christoph; Wedding, Ulrich; Sträter, Jörn; Limberg, Bernd; Stremmel, Wolfgang

doi:10.1111/j.1365-2796.1997.tb00013.x

Cited by 19 publications

(7 citation statements)

References 10 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Side effects included mild depression and biochemical hypothyroidism treated with supplemental levothyroxine [135]. Taken together, these, and other reports [113, 115,] suggest a synergistic effect of IFN-α and corticosteroids when used together in the treatment of SMCD.…”

Section: Interferon Treatment Of Systemic Mastocytosissupporting

confidence: 56%

“…‘Near absence’ of ascites and hepatomegaly after 12 months of treatment at up to 5 × 10 6 U daily were noted in the initial report of Kluin-Nelemans et al [104]. Subsequently, in numerous reports, the effectiveness of IFN-α to improve gastrointestinal symptoms such as diarrhea, vomiting, and cramping [108, 113], to effect resolution of ascites, decrease hepatomegaly [105, 108], and reduce size and number of intra-abdominal lymphadenopathy [105] have all been observed. However, these clinical benefits may also occur in the absence of any reduction in bone marrow mast cell infiltration, and reduction in intra-abdominal lymphadenopathy is not consistently reported [108].…”

Section: Interferon Treatment Of Systemic Mastocytosismentioning

confidence: 99%

“…In many series the follow-up duration is less than 1 year [116]. The prolonged benefit reported in several series (3 years [113]; 5 years [112]; 3 years [114]; 22 months [108]; 25 months [115)] is commonly associated with higher doses of IFN-α. Commonly in these series individual doses of IFN-α exceed 4.5 × 10 6 U and the frequency of administration is at least 3 times per week.…”

Section: Interferon Treatment Of Systemic Mastocytosismentioning

confidence: 99%

“…Using this approach, decrease [103, 113, 115, 121] or normalization [109] of serum levels of histamine, or decreased urinary excretion of histamine [103, 108, 113], the histamine metabolite methyl imidazole acetic acid (MIAA) [105, 122], and both N-methyl histamine and MIAA [104, 123] have been reported in patients followed from several months to more than 1 year.…”

Section: Interferon Treatment Of Systemic Mastocytosismentioning

confidence: 99%

“…Commonly, on the low end of the treatment range doses of from 3 to 5 × 10 6 U 3 times per week [107,108,109] are employed, while doses of from 10 × 10 6 U 3 times per week [110] to 4.5–8.0 × 10 6 U daily are found at the high end of the dosing range [103, 111, 112]. When interferons have been employed in combination with prednisone, doses of IFN-α2B of 5 ×10 6 U 3 times per week [113] to 3 × 10 6 U daily [114] have been used, while for IFN-α2A a dose of 6 × 10 6 U 3 times per week has been reported [115]. …”

Section: Interferon Treatment Of Systemic Mastocytosismentioning

confidence: 99%

See 4 more Smart Citations

Interferon Treatment for Hypereosinophilic Syndromes and Systemic Mastocytosis

Butterfield¹

2005

Acta Haematol

View full text Add to dashboard Cite

Hypereosinophilic syndromes (HES) and systemic mastocytosis (SMCD) are heterogeneous disorders with clinical symptoms from local and remote effects of excessive proliferation of eosinophils and mast cells, respec tively. Interferon α (IFN-α), alone or in combination with other medications, can be a useful, and at times life-saving, treatment for patients with HES. Receptors for IFN-α are present on eosinophils, and clinical benefits are due to its effect on eosinophil proliferation, migration, activation, and survival. These effects are likely mediated through multiple pathways including, but not limited to, inhibition of eosinophil colony-forming cells, upregulation of IFN-γ synthesis, and inhibition of production of eosinophil-active cytokines by T cells, mast cells, and mononuclear cells. IFN-α has been life-saving for patients with intractable HES that were resistant to prednisone, hydroxyurea, and other agents. Resistance to the eosinopenic effect of IFN-α does not develop and the dose of IFN-α necessary to maintain control of eosinophilia often decreases with time. The combination of IFN-α and hydroxyurea is very useful and allows dosage reduction of IFN-α and better control of hypereosinophilia than with either agent alone. The efficacy of IFN-α for treatment of SMCD has been more difficult to establish, with both favorable and unfavorable results reported. The disparate results may have resulted from the small number of patients with SMCD treated with IFN-α, the use of various criteria for a ‘successful’ treatment outcome, short duration of treatment and follow-up, and the use of modest dosages. In reported series, side effects from IFN-α have frequently been dose-limiting. IFN-α improves many of the clinical symptoms of SMCD including dermatological, hematological, gastrointestinal, and systemic symptoms associated with histamine release. IFN-α has a beneficial effect on skeletal symptoms because of its ability to increase bone density and reduce painful episodes from vertebral fractures. No consistent improvement in bone marrow infiltration by mast cells has been demonstrated except in a recent study employing high dosages of IFN-α. A beneficial effect from the combination of IFN-α and prednisone has been reported for several patients, suggesting that combined use of these two medications may provide synergism in treatment outcomes.

show abstract

Section: Interferon Treatment Of Systemic Mastocytosissupporting

confidence: 56%

Section: Interferon Treatment Of Systemic Mastocytosismentioning

confidence: 99%

Section: Interferon Treatment Of Systemic Mastocytosismentioning

confidence: 99%

Section: Interferon Treatment Of Systemic Mastocytosismentioning

confidence: 99%

Section: Interferon Treatment Of Systemic Mastocytosismentioning

confidence: 99%

See 3 more Smart Citations

Interferon Treatment for Hypereosinophilic Syndromes and Systemic Mastocytosis

Butterfield¹

2005

Acta Haematol

View full text Add to dashboard Cite

show abstract

Urticaria and Mastocytosis

Grattan¹,

Black²

2004

Rook's Textbook of Dermatology

View full text Add to dashboard Cite

Mastocytosis

Grattan,

Radia

2016

Rook's Textbook of Dermatology, Ninth Edition

View full text Add to dashboard Cite

Mastocytosis is a rare multisystem disorder of increased mast cell numbers in the skin and other tissues. Molecular genetic analysis of bone marrow reveals a mutation of KIT in nearly all adult patients with systemic disease. Although mastocytosis is primarily a haematological disorder, it often presents with skin lesions to dermatologists or paediatricians. Patients may also present with symptoms or complications of the disease to allergists, metabolic bone disease specialists or accident and emergency departments. A diagnosis of mastocytosis can be confirmed by lesional skin biopsy. Systemic mastocytosis is usually diagnosed by bone marrow biopsy. Local or systemic symptoms from mast cell mediator release are common. Complications of adult disease include anaphylaxis, osteoporosis and the lifetime development of associated haematological non‐mast cell diseases. At least 50% of children will grow out of mastocytosis by puberty, particularly those with early‐onset disease. Treatment is aimed primarily at symptom control with antimediator drugs, including H1 antihistamines, but patients with advanced systemic disease may require cytoreductive drugs.

show abstract

Clinical stable systemic mastocytosis with interferon alpha 2b therapy.

Abstract: Hubner C, Wedding U, Strater J, Limberg B, established therapy. We describe a patient with

Cited by 19 publications

References 10 publications

Interferon Treatment for Hypereosinophilic Syndromes and Systemic Mastocytosis

Interferon Treatment for Hypereosinophilic Syndromes and Systemic Mastocytosis

Urticaria and Mastocytosis

Mastocytosis

Contact Info

Product

Resources

About