Phase II study of imatinib mesylate as therapy for patients with systemic mastocytosis

Vega-Ruiz, Arturo; Cortes, Jorge; Sever, Matjaž; Manshouri, Taghi; Quintás‐Cardama, Alfonso; Luthra, Rajyalakshmi; Kantarjian, Hagop; Verstovšek, Srđan

doi:10.1016/j.leukres.2008.12.020

Cited by 149 publications

(111 citation statements)

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“…In yet another study of 20 SM patients treated with IM, only one KITD816V-negative patient responded while six other patients reported symptomatic improvement [110]. Finally, in another study wherein 17 SM patients received IM treatment, the response rate was 29% (one complete and four partial remissions), all in KITD816V-negative patients [111].…”

Section: Imatinib Mesylate (Im)mentioning

confidence: 89%

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: Imatinib Mesylate (Im)mentioning

confidence: 89%

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

Pardanani

2015

American J Hematol

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“…10 O nosso caso tem a particularidade de apresentar uma mutação patogénica D816V do c-kit em todas as linhagens medulares e não apenas na linhagem mastocitária, fator que se associa a maior risco de evolução de forma indolente para forma agressiva de MS. 17,18 Esta mutação especí-fica, ativadora de c-kit, está presente em mais de 80% dos adultos com MS e confere resistência ao tratamento com os inibidores das cinases de tirosina, imatininb e dasatinib. 5,19 Não existe, até ao momento, nenhuma terapia capaz de alterar o curso natural da MC, nomeadamente da TMEP. 20,21 Assim, a abordagem terapêutica passa pela evicção dos fatores desencadeantes da desgranulação mastocitária e tratamento sintomático.…”

Section: 11unclassified

Um Novo Olhar sobre a Telangiectasia Macularis Eruptiva Perstans

Pinho

Cardoso

Oliveira

et al. 2016

SPDV

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RESUMO -A telangiectasia macularis eruptiva perstans (TMEP) é uma forma rara de mastocitose cutânea com semiologia e histologia próprias, mas não isenta de controvérsia em relação à sua individualização enquanto entidade nosológica. Para alguns autores, a presença exclusivamente de lesões maculosas telangiectásicas a par de dilatação do plexo vascular superficial e de infiltrados mastocitários pouco densos na derme superficial é suficiente para o diagnóstico de TMEP. Ainda assim, em algumas classificações vigentes esta entidade não é considerada separadamente de outras formas de mastocitose cutânea, pois em muitos casos com lesões telangiectásicas também se podem observar lesões de urticária pigmentosa. Nós apresentamos um caso de mastocitose cutânea com características clínicas, dermatoscópicas e histológicas compatíveis com TMEP, de aparecimento na oitava dé-cada de vida e sem outros sintomas de ativação mastocitária, além do prurido. Porém, o estudo complementar revelou a presença concomitante de mastocitose sistémica indolente. Adicionalmente, revimos a evidência mais recente a favor do reconhecimento e individualização desta entidade. PALAVRAS-CHAVE -Mastocitose Cutânea; Telangiectasia; Urticária Pigmentosa. A New Insight over Telangiectasia Macularis Eruptiva Perstans ABSTRACT -Telangiectasia macularis eruptiva perstans (TMEP) is a rare form of cutaneous mastocytosis with distinctive semiology and histopathology, but that is not free of controversy regarding its individualization as a nosological entity. For some authors, the presence of exclusively telangiectatic macules, along with superficial vascular plexus dilation and low density of mast cell infiltrates in the upper

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“…It has been found to be effective in relieving symptoms, and there is an improvement in cytopenias, organomegaly and serum tryptase levels [6]. However, most responses are transient.…”

Section: S110mentioning

confidence: 99%

Systemic Mastocytosis with Associated Clonal Hematological Non-Mast Cell Lineage Disorder (MDS-RCMD): A Difficult Disease to Diagnose and Treat

Ravichandran

Chitrapur

Bhave

et al. 2015

Indian J Hematol Blood Transfus

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Systemic mastocytosis is a rare and recalcitrant disorder with nonspecific clinical features. Hence, a high index of suspicion is required. Here, we report the case of a 64 years old male presenting with chronic diarrhoea that was evaluated at different centres and treated with multiple lines of therapy. The diagnosis of aggressive systemic mastocytosis was finally clinched following a holistic work up that included a Jejunal biopsy and a laparoscopic lymph node biopsy. Treatment of this disorder is difficult, responses are transient and most patients will eventually relapse, as illustrated by this case. Cladribine, Interferon a, steroids and imatinib have limited success in the management of this disease. The role of stem cell transplant is uncertain.

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Phase II study of imatinib mesylate as therapy for patients with systemic mastocytosis

Cited by 149 publications

References 10 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

Um Novo Olhar sobre a Telangiectasia Macularis Eruptiva Perstans

Systemic Mastocytosis with Associated Clonal Hematological Non-Mast Cell Lineage Disorder (MDS-RCMD): A Difficult Disease to Diagnose and Treat

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