Myelomastocytic leukemia versus mast cell leukemia versus systemic mastocytosis associated with acute myeloid leukemia: A diagnostic challenge

Arredondo, Angela R.; Gotlib, Jason; Shier, Luke; Medeiros, Bruno C.; Wong, Kathleen; Cherry, Athena M.; Corless, Christopher L.; Arber, Daniel A.; Valent, Peter; George, Tracy I.

doi:10.1002/ajh.21713

Cited by 49 publications

(45 citation statements)

References 27 publications

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“…About 80% of patients with mastocytosis have findings of skin involvement. In systemic mastocytosis the bone marrow is almost always involved [1].…”

Section: Discussionmentioning

confidence: 99%

“…While Indolent SM must have one or more "B" findings, 2 or more "B" findings are required for diagnosing SM. ASM and MCL are required one or more "C" findings [1].…”

Section: Discussionmentioning

confidence: 99%

“…Additional clonal myeloid disease does not exist. MML is a rare disease that has previously been described in patients with advanced myeloid neoplasms (most commonly refractory anemia with excess blasts, a subtype of MDS, or AML) with elevated numbers of immature atypical mast cells who do not meet full criteria for SM [1,4,8,9,10]. MML typically presents with an increase in myeloblasts (>5%) as well as >10% metachromatic blasts in the peripheral blood and/or bone marrow but without focal dense mast cell infiltrates, without mast cell expression of CD2 or CD25 and without evidence of the D816V KIT mutation by PCR (Table1).…”

Section: Discussionmentioning

confidence: 99%

“…The 2008 WHO classification of SM recognizes four major subtypes: Indolent SM, SM with associated clonal hematologic non-mast cell lineage disease (SM-AHNMD), aggressive SM (ASM), and mast cell leukemia (MCL). Additionally, the differential diagnosis of advanced systemic mast cell neoplasms also includes myelomastocytic leukemia (MML) [1].…”

Section: Introductionmentioning

confidence: 99%

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Variants of systemic mastocytosis: A report of three cases

2015

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“…About 80% of patients with mastocytosis have findings of skin involvement. In systemic mastocytosis the bone marrow is almost always involved [1].…”

Section: Discussionmentioning

confidence: 99%

“…While Indolent SM must have one or more "B" findings, 2 or more "B" findings are required for diagnosing SM. ASM and MCL are required one or more "C" findings [1].…”

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Variants of systemic mastocytosis: A report of three cases

2015

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“…Другой подтип МЦ, ранее не включенный в классификацию ВОЗ, -миеломастоцитарный лейкоз, который представляет ред-кое заболевание, описано у пациентов с развернутой стадией ми-елоидных заболеваний (например, рефрактерная анемия с из-бытком бластов и острый миелоидный лейкоз), характеризуется наличием большого количества атипичных ТК, отсутствием кри-териев СМЦ, увеличенным количеством миелобластов (>5%), очаговой инфильтрацией ТК, экспрессирующими CD25 и CD2, наличием мутации D816VKIT [20,21].…”

Section: терапевтический архив 12 2014unclassified

Mastocytosis. Review of the literature and description of clinical cases

Меликян¹,

Subortseva²,

Горячева³

et al. 2014

Ter. arkh.

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Systemic mastocytosis with associated clonal hematological non‐mast‐cell lineage disease: A case review

Üstün¹,

Savage²,

Gotlib³

et al. 2011

American J Hematol

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A 54-year-old male was originally referred to our institution 2 years prior with a diagnosis of systemic mastocytosis (SM). Past medical history was significant for urticaria pigmentosa, which was diagnosed 15 years prior, and fatigue, diarrhea, and weight loss (10%), which were more recent in nature. In addition, a recent computed tomography (CT) scan revealed lymphadenopathy and hepatosplenomegaly.Mastocytosis is currently defined, according to the 2008 WHO, as a clonal, neoplastic proliferation of mast cells (MCs) in one or multiple organs [1][2][3]. Clinical symptoms are divided into four separate categories, including (1) constitutional symptoms (i.e., fever, fatigue, and weight loss), (2) skin manifestations (such as urticaria pigmentosa), (3) mediator-related symptoms (to include abdominal pain, flushing, headache, hypotension, tachycardia among others), and (4) musculoskeletal complaints. These are caused by the release of chemical mediators such as histamine, eicosanoids, proteases, and heparin and also by infiltration of tissues by the neoplastic MCs.In cutaneous mastocytosis, MC infiltration is limited to the skin with a typical presentation in childhood. Urticaria pigmentosa is a macroscopic description of the tendency for the lesions to urticate (sting and blister) and to accumulate intraepidermal pigment. In children, cutaneous mastocytosis typically has a benign clinical course and may regress spontaneously [4]. However, in adults, cutaneous disease is often associated with indolent systemic involvement and skin lesions do not typically regress. The WHO now recognizes three major variants of cutaneous mastocytosis, urticaria pigmentosa/maculopapular cutaneous mastocytosis, diffuse cutaneous mastocytosis, and mastocytoma of skin.SM is diagnosed when one major criterion and one minor criterion or at least three minor criteria are present. The major diagnostic criterion for SM is fulfilled by the presence of multifocal dense MC infiltrates (15 MCs per aggregate) detected histologically in the bone marrow or in another extracutaneous organ(s). Minor criteria include: (1) 25% of the MCs in the infiltrate are spindle-shaped or show atypical morphology or, 25% are immature or atypical on BM aspirate smears; (2) a serum tryptase level is persistently greater than 20 ng/mL (this parameter is not valid if there is an associated clonal myeloid disorder); (3) detection of the characteristic D816V KIT mutation and (4) expression of CD2, CD25, or both in neoplastic MCs [1][2][3][4][5]. In these patients, splenomegaly may be appreciated, but is often minimal. However, lymphadenopathy and hepatomegaly are much rarer but were noted in our patient.A complete blood count, at the time of referral, showed a normal hemoglobin (14.6 g/dL) and platelet count (326 3 10 9 /L), and a leukocytosis (17.1 3 10 9 /L) with increased monocytes (absolute monocytes: 1.4 3 10 9 /L). A lymphocytosis was not present, and his monocytosis had lasted more than 3 months. The bone marrow (BM) aspiration/biopsy slides revealed a normocellula...

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Myelomastocytic leukemia versus mast cell leukemia versus systemic mastocytosis associated with acute myeloid leukemia: A diagnostic challenge

Cited by 49 publications

References 27 publications

Variants of systemic mastocytosis: A report of three cases

Variants of systemic mastocytosis: A report of three cases

Mastocytosis. Review of the literature and description of clinical cases

Systemic mastocytosis with associated clonal hematological non‐mast‐cell lineage disease: A case review

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