Systemic mastocytosis associated with chronic myelomonocytic leukemia: Clinical features and response to interferon alfa therapy

Petit, Antoine; Pulik, M; Gaulier, A; Lionnet, François; Mahé, A.; Sigal, Michèle

doi:10.1016/0190-9622(95)91545-1

Cited by 47 publications

(32 citation statements)

References 7 publications

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“…IFN-a is often considered the first-line cytoreductive therapy in symptomatic SM; since the initial report in 1992 [79], several case reports or small series have shown IFN-a (IFN-a2b in most instances) to improve symptoms of MC degranulation, decrease BM MC infiltration, and ameliorate mastocytosis-related ascites/hepatosplenomegaly, cytopenias, skin findings, and osteoporosis [80][81][82][83][84][85][86][87][88][89][90][91][92]. IFN-a treatment is not uniformly effective [93], and the frequency of major response (i.e., complete resolution of one or more baseline 'C' findings) is 20-30%; the optimal dose and duration of IFN-a therapy for SM remain unclear, however concurrent administration of corticosteroids (prednisone) may improve its efficacy (up to 40% major response rate) and tolerability [87,94].…”

Section: Interferon (Ifn)-amentioning

confidence: 99%

Systemic mastocytosis in adults: 2015 update on diagnosis, risk stratification, and management

Pardanani

2015

American J Hematol

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Disease overview: Systemic mastocytosis (SM) results from a clonal proliferation of abnormal mast cells (MC) in one or more extracutaneous organs.Diagnosis: The major criterion is presence of multifocal clusters of morphologically abnormal MC in the bone marrow. Minor diagnostic criteria include elevated serum tryptase level, abnormal MC expression of CD25 and/or CD2, and presence of KITD816V.Risk stratification: The 2008 World Health Organization classification of SM has been shown to be prognostically relevant. Classification of SM patients into indolent SM (ISM), aggressive SM (ASM), SM associated with a clonal non‐MC lineage disease (SM‐AHNMD), and mast cell leukemia (MCL) subgroups is a useful first step in establishing prognosis.Management: SM treatment is generally palliative. ISM patients have a normal life expectancy and receive symptom‐directed therapy; infrequently, cytoreductive therapy may be indicated for refractory symptoms. ASM patients have disease‐related organ dysfunction; interferon‐α (+/−corticosteroids) can control dermatological, hematological, gastrointestinal, skeletal, and mediator‐release symptoms, but is hampered by poor tolerability. Similarly, cladribine has broad therapeutic activity, with particular utility when rapid MC debulking is indicated; the main toxicity is myelosuppression. Imatinib has a therapeutic role in the presence of an imatinib‐sensitive KIT mutation or in KITD816‐unmutated patients. Treatment of SM‐AHNMD is governed primarily by the non‐MC neoplasm; hydroxyurea has modest utility in this setting; there is a role for allogeneic stem cell transplantation in select cases.Investigational Drugs: Recent data confirms midostaurin's significant anti‐MC activity in patients with advanced SM. Am. J. Hematol. 90:251–262, 2015. © 2015 Wiley Periodicals, Inc.

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Section: Interferon (Ifn)-amentioning

confidence: 99%

Systemic mastocytosis in adults: 2015 update on diagnosis, risk stratification, and management

Pardanani

2015

American J Hematol

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“…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%

“…For patients in numerous other reports thrombocytopenia has necessitated dosage reduction of: 5 × 10 6 U daily to 3 times per week [104];6 × 10 6 U daily to 4.5 × 10 6 U 3 times per week [108]; and 5 ×10 6 U daily to 3 × 10 6 U daily [118]. Increased hair loss and weight loss resolved with a change in frequency from 5 × 10 6 U 5 times per week to 3 times per week in a series of patients with urticaria pigmentosa [109].…”

Section: Interferon Treatment Of Systemic Mastocytosismentioning

confidence: 99%

“…Both IFN-α2A (Roferon) [106] and IFN-α2B have been employed at similar doses. 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%

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Interferon Treatment for Hypereosinophilic Syndromes and Systemic Mastocytosis

Butterfield¹

2005

Acta Haematol

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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.

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“…Although there is no established treatment for this disease, some studies (2)(3)(4)(5)(6)(7) have suggested the usefulness ofinterferonoc. Here, we describe a case of systemic mastocytosis with polypoid lesions in the colon successfully treated with interferon-a-2b.…”

Section: Introductionmentioning

confidence: 99%

Systemic Mastocytosis with Extensive Polypoid Lesions in the Intestines; Successful Treatment with Interferon-.ALPHA..

Takasaki

Tsukasaki

et al. 1998

Intern. Med.

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A 35-year-old female presented in 1989 with hepatosplenomegaly, but no conclusive diagnosis was established. From 1992, she experienced transient episodes of facial flushing and palpitations. Osteosclerotic change was detected radiologically. Colonoscopy revealed massive polypoid lesions. Mast cells were demonstrated in bone marrow smear and imprinted preparations of colon biopsy specimens by toluidine blue staining. Plasmaconcentrations of histamine and soluble c-kit were elevated. She was successfully treated with interferon-a and prednisolone, resulting in the disappearance of histamine-related attacks and a gradual decrease in tumor size. However, the remission was interferon dose dependent. This case was considered as systemic mastocytosis with massive polypoid colon lesions and showed the importance of maintenance therapy with interferon-a. (Internal Medicine 37: 484-488, 1998)

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Systemic mastocytosis associated with chronic myelomonocytic leukemia: Clinical features and response to interferon alfa therapy

Cited by 47 publications

References 7 publications

Systemic mastocytosis in adults: 2015 update on diagnosis, risk stratification, and management

Systemic mastocytosis in adults: 2015 update on diagnosis, risk stratification, and management

Interferon Treatment for Hypereosinophilic Syndromes and Systemic Mastocytosis

Systemic Mastocytosis with Extensive Polypoid Lesions in the Intestines; Successful Treatment with Interferon-.ALPHA..

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