Hyperfibrinolysis in a case of myelodysplastic syndrome with leukemic spread of mast cells

Wimazal, Friedrich; Sperr, Wolfgang R.; Horny, Hans‐Peter; Carroll, Veronica A.; Binder, Bernd R.; Fonatsch, Christa; Walchshofer, Sabine; Födinger, Manuela; Schwarzinger, Ilse; Samorapoompichit, Puchit; Chott, Andreas; Dvorak, Ann M.; Lechner, Klaus; Valent, Peter

doi:10.1002/(sici)1096-8652(199905)61:1<66::aid-ajh12>3.0.co;2-3

Cited by 24 publications

(41 citation statements)

References 54 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Patients receiving cytoreductive treatment and supportive care had a short survival (1). In one case with myelomastocytic leukemia, intensive polychemotherapy was administered and resulted in CR (2). Based on this knowledge and the age of our patient, we decided to apply intensive chemotherapy and stem cell transplantation.…”

Section: Discussionmentioning

confidence: 97%

See 1 more Smart Citation

Myelomastocytic Leukemia: Evidence for the Origin of Mast Cells from the Leukemic Clone and Eradication by Allogeneic Stem Cell Transplantation

Sperr¹,

Drach²,

Hauswirth³

et al. 2005

Clinical Cancer Research

View full text Add to dashboard Cite

Purpose: Myelomastocytic leukemia is a term used for patients with advanced myeloid neoplasms, in whom elevated numbers of immature atypical mast cells are found, but criteria for a primary mast cell disease are not met. The origin of mast cells in these patients is presently unknown. Patient and Methods: We have analyzed clonality of mast cells in an 18-year-old patient suffering from acute myeloid leukemia with a complex karyotype including a t(8;21) and mastocytic transformation with a huge increase in immature mast cells and elevated serum tryptase level, but no evidence for a primary mast cell disease/mastocytosis. Results: As assessed by in situ fluorescence hybridization combined with tryptase staining, both the tryptase-negative blast cells and the tryptase-positive mast cells were found to contain the t(8;21)-specific AML1/ETO fusion gene. Myeloablative stem cell transplantation resulted in complete remission with consecutive disappearance of AML1/ETO transcripts, decrease of serum tryptase to normal range, and disappearance of neoplastic mast cells. Conclusion: These data suggest that mast cells directly derive from the leukemic clone in patients with myelomastocytic leukemia.Myelomastocytic leukemia is a term used for patients with advanced myeloid neoplasms, in whom a substantial increase in immature atypical mast cells is found, but the criteria for a primary mast cell disease (mast cell leukemia or systemic mastocytosis) are not fulfilled (1 -5). Typically, patients with myelomastocytic leukemia exhibit an increase in blast cells as well as >10% metachromatic cells in peripheral blood and/or bone marrow smears (3 -5). Blast cells are myeloblasts by morphologic and immunophenotypic criteria. These patients are thus diagnosed to have an advanced myelodysplastic syndrome with excess of blasts, a myeloproliferative disease, or an acute myeloid leukemia (AML; refs. 1 -4). Myelomastocytic leukemia is a rare disease. In fact, <5% of all patients with myelodysplastic syndrome, myeloproliferative disease, or AML are considered to develop a ''mastocytic transformation '' (3, 4).Metachromatic cells in myelomastocytic leukemia are immature, often exhibit a blast-like morphology, and are mast cell lineage cells by electron microscopic and immunophenotypic criteria (CD117 + , tryptase + , CD11b À , and CD123 À ; refs. 3, 4). Mature mast cells may be seen occasionally. As in other patients with myelodysplastic syndrome, myeloproliferative disease, or AML, major signs of dysplasia may be found in erythroid and granulomonocytic cells (1 -3). The bone marrow histology shows a diffuse spread of metachromatic cells (1 -6), whereas multifocal aggregates of tryptase-positive mast cells, typically seen in patients with systemic mastocytosis (6, 7), are not found (1 -6). Other criteria of systemic mastocytosis (6) are also not met. In fact, mast cells are CD2 negative and CD25 negative and do not exhibit transforming mutations at codon 816 of c-kit (1 -6). Thus, a number of diagnostic criteria are available that ...

show abstract

Section: Discussionmentioning

confidence: 97%

“…Similar to ''true'' mast cell leukemia, most patients survive only a few months (1 -6). However, complete remission (CR) in response to polychemotherapy has been reported (2). This is of particular interest, because patients with mast cell leukemia or aggressive systemic mastocytosis are usually not entering CR in response to polychemotherapy (6, 10, 12 -15).…”

mentioning

confidence: 99%

Myelomastocytic Leukemia: Evidence for the Origin of Mast Cells from the Leukemic Clone and Eradication by Allogeneic Stem Cell Transplantation

Sperr¹,

Drach²,

Hauswirth³

et al. 2005

Clinical Cancer Research

View full text Add to dashboard Cite

show abstract

“…The term myelomastocytic leukemia was first proposed in 1999 by Wimazal et al 8 Valent et al 6 formally described the current criteria used for the diagnosis of myelomastocytic leukemia in 2002. In earlier studies, however, others had reported on several cases that, in retrospect, met the current diagnostic criteria for myelomastocytic leukemia.…”

Section: Discussionmentioning

confidence: 99%

“…2,6,17,18 A search of the English-language literature identified only 12 reported cases of myelomastocytic overlap syndrome, including 9 patients with myelomastocytic leukemia. 5,[7][8][9][10]19,20 The clinical and laboratory data for reported cases of myelomastocytic overlap syndrome are listed in Table 1. One patient had blast phase chronic myelogenous leukemia, 2 patients had a myelodysplastic syndrome with mastocytic differentiation, and 9 patients had true myelomastocytic leukemia.…”

Section: Discussionmentioning

confidence: 99%

“…In contrast with patients with SM, urticaria pigmentosa-like skin lesions have never been described in any patient with myelomastocytic leukemia reported to date. [5][6][7][8][9][10] Myelomastocytic leukemia is distinct from SM with associated clonal hematologic nonemast cell lineage disease (SM-AHNMD), mast cell leukemia, and AML with tryptase expression, KIT (v-kit Hardy-Zuckerman 4 feline sarcoma viral oncogene homolog; c-KIT gene) mutation at codon 816, or both. 5,[11][12][13][14][15][16] This report presents a patient with myelomastocytic leukemia that had aberrant CD25 expression.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

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

Rich

Sun

Aldayel

et al. 2014

Clinical Lymphoma Myeloma and Leukemia

View full text Add to dashboard Cite

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

et al. 2010

View full text Add to dashboard Cite

Hyperfibrinolysis in a case of myelodysplastic syndrome with leukemic spread of mast cells

Cited by 24 publications

References 54 publications

Myelomastocytic Leukemia: Evidence for the Origin of Mast Cells from the Leukemic Clone and Eradication by Allogeneic Stem Cell Transplantation

Myelomastocytic Leukemia: Evidence for the Origin of Mast Cells from the Leukemic Clone and Eradication by Allogeneic Stem Cell Transplantation

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

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

Contact Info

Product

Resources

About