Concurrent acute myeloid leukemia and systemic mastocytosis

Wong, K. F.; Chan, Jmm; Chan, Jane; Kwong, Yok–Lam; K., S.; Chow, Tsz-Kit

doi:10.1002/ajh.2830380319

Cited by 30 publications

(21 citation statements)

References 9 publications

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“…9 However, our patient is still in complete haematological remission after two years of follow up, although the polychemotherapy was much more effective (and gave a much faster response) on the AML than the mastocytosis, which persisted morphologically for a period of at least 18 months, whereas the AML showed immediate complete remission after induction phase chemotherapy. In this respect, our case confirms the experiences reported by others, [17][18][19] and underlines the resistance of neoplastic mast cells to conventional chemotherapy. The delayed decrease of mast cell infiltration in response to chemotherapy may be explained by the fact that treatment resulted in the depletion of mast cell progenitors but not of mature mast cells.…”

Section: Discussionsupporting

confidence: 92%

Acute myeloid leukaemia with t(8;21) associated with “occult” mastocytosis. Report of an unusual case and review of the literature

Sotlar²,

Lorenzen³

et al. 2004

J Clin Pathol

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Approximately 20% of patients with systemic mastocytosis (SM) have an associated haematological, clonal, non-mast cell lineage disease, and most exhibit an associated myelogenous neoplasm. This report describes a 48 year old man with acute myeloid leukaemia (AML) and a type t(8;21) cytogenetic abnormality. Associated bone marrow mastocytosis (a defined subtype of SM) was only detected after successful polychemotherapy in the state of bone marrow aplasia, and persisted after complete remission of AML. The diagnosis of mastocytosis was based on the demonstration of a multifocal dense mastocytic infiltrate. The atypical mast cells showed prominent spindling and an aberrant immunophenotype, with coexpression of tryptase, chymase, KIT, and CD25—which is expressed only on neoplastic (not normal) mast cells. In addition, the transforming somatic mutation D816V of the c-kit gene was detected. Re-examination of the pretherapeutic (initial) bone marrow revealed a slight diffuse increase in partially spindle shaped mast cells also exhibiting an abnormal immunophenotype, with CD25 expression, although compact mastocytic infiltrates were not detected. Because the D816V mutation was detected in the initial bone marrow specimen, strict application of three minor diagnostic criteria (spindling, CD25, D816V) enabled a diagnosis of SM-AML to be confirmed retrospectively in the initial bone marrow tissue.

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Section: Discussionsupporting

confidence: 92%

Acute myeloid leukaemia with t(8;21) associated with “occult” mastocytosis. Report of an unusual case and review of the literature

Sotlar²,

Lorenzen³

et al. 2004

J Clin Pathol

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“…In our report, two of three such patients achieved complete remission from both SM and the associated hematologic disorder: one with idarubicin and high-dose cytarabine therapy for concurrent AML, the other with allogeneic SCT for concurrent MDS. In general, evidence suggests that chemotherapy induces relatively short remissions, with a greater effect on the non-mast cell malignancy and little or no effect on the SM [14,33]. The prognosis of these patients is relatively poor [34].…”

Section: Discussionmentioning

confidence: 99%

Management of patients with systemic mastocytosis: Review of M. D. Anderson Cancer Center experience

et al. 2004

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Mastocytosis is characterized by mast cell proliferation that may be limited to the skin (cutaneous mastocytosis) or may involve one or more extracutaneous organs, e.g., the bone marrow (systemic mastocytosis; SM). This study objective is to evaluate the features and outcome of patients referred to M. D. Anderson Cancer Center (MDACC) with SM. A search of the MDACC database from 1944 to 2002 was conducted for patients with SM and review of their clinical charts. Eighteen patients with mastocytosis were identified in the MDACC database; 15 (11 males and 4 females) had SM and available information. Two had associated myelodysplastic syndrome (MDS), and one had acute myeloid leukemia (AML). The median age was 58 years (range 31-80). Nine patients were treated with subcutaneous interferon-alpha, and only 1 experienced temporary control of the disease. Three of these patients were then treated with imatinib mesylate: transient improvement was noted in two patients. One patient underwent stem cell transplantation as first therapy and achieved complete remission; this patient had associated MDS and is now in complete remission for 8 years. The patient with associated AML was treated with high-dose cytarabine and idarubicin; he has been in complete remission for 16 months. One patient was treated with induction chemotherapy consisting of high-dose cytarabine and 2CDA but expired due to sepsis. Three patients received symptomatic therapy only; these were the only 3 patients who presented with normal blood counts. SM is rare and has no effective standard of care. Collaboration among academic centers to accrue enough patients to evaluate novel therapeutic strategies is needed. Am.

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“…Morphologic examination of BM specimens dictates a broad differential diagnosis that includes acute promyelocytic leukemia (the referring diagnosis in the case presented), acute Myelomastocytic Leukemia e176 -Clinical Lymphoma, Myeloma & Leukemia October 2014 basophilic leukemia, tryptase-positive AML, acute monocytic leukemia, mast cell leukemia, SM-AHNMD, chronic myelomonocytic leukemia, and a basophilic variant of blast phase of chronic myelogenous leukemia. 11,14,22,23 Metachromatic features of mast cells on Giemsa stains and distinct immunophenotypes of immature mast cells (CD117-positive, tryptase-positive, CD11b-negative, and CD123-negative) are extremely helpful in diagnosing myelomastocytic leukemia. 2,17,20 Clinically, patients with myelomastocytic overlap syndrome differ from patients with mast cell leukemia, who often have systemic mast cell disease before the onset of mast cell leukemia.…”

Section: Discussionmentioning

confidence: 99%

Myelomastocytic Leukemia With Aberrant CD25 Expression: Case Report and Review of the Literature

Rich

Sun

Aldayel

et al. 2014

Clinical Lymphoma Myeloma and Leukemia

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Concurrent acute myeloid leukemia and systemic mastocytosis

Cited by 30 publications

References 9 publications

Acute myeloid leukaemia with t(8;21) associated with “occult” mastocytosis. Report of an unusual case and review of the literature

Acute myeloid leukaemia with t(8;21) associated with “occult” mastocytosis. Report of an unusual case and review of the literature

Management of patients with systemic mastocytosis: Review of M. D. Anderson Cancer Center experience

Myelomastocytic Leukemia With Aberrant CD25 Expression: Case Report and Review of the Literature

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