Malignant mastocytosis with circulating mast cells

Torrey, E. H.; Simpson, Karen M.; Wilbur, Stephen; Muñoz, Phillip A.; Skikne, Barry S.

doi:10.1002/ajh.2830340409

Cited by 26 publications

(10 citation statements)

References 16 publications

(10 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…reported on the relationship between the extent of bone marrow infiltration by mast cells and the prognosis: patients with >20% of mast cells generally have more rapid disease progression than those with less 13 . It has also been noted that, regardless of the percentage of circulating mast cells, extensive involvement of the bone marrow with neoplastic mast cells should be considered to be a sign of aggressive disease in malignant mastocytosis 6 …”

Section: Discussionmentioning

confidence: 99%

“…Mast cell leukemia is an extremely rare form of systemic mastocytosis, with an incident of only 1% among adult patients with systemic mastocytosis in a recent retrospective study 4 . This subtype is characterized by severe bone marrow involvement, appearance of circulating mast cells in the peripheral blood, multiple organ dysfunction, and an aggressive clinical course 5–7 . The number of circulating neoplastic mast cells in the peripheral blood varies from case to case in mast cell leukemia and, if the mast cells comprise <10% of the circulating nucleated cells, it is called the aleukemic variant 2,8 …”

mentioning

confidence: 99%

See 1 more Smart Citation

Mast cell leukemia with rapidly progressing portal hypertension

Yoshida

Nishikawa²,

Yamamoto³

et al. 2009

Pathology International

View full text Add to dashboard Cite

Reported herein is an autopsy case of mast cell leukemia, a rare form of systemic mastocytosis, complicated with portal hypertension. A 52-year-old woman presented with urticaria-like skin symptoms, anemia, and thrombocytopenia. Atypical mast cells (CD2+, CD25+, CD117+) with toluidine blue metachromasia were found in the peripheral blood and on bone marrow aspiration smears. Chemotherapy with cytosine arabinoside and idarubicin was ineffective and the patient died of multi-organ failure with rapidly progressing hepatosplenomegaly and large-volume ascites 3 months after admission. At autopsy the bone marrow, spleen, liver, and lymph nodes were extensively infiltrated by atypical tumor cells with occasional bi- or multi-lobated nuclei. They were positive for mast cell tryptase and possessed an activating mutation of the c-kitgene (D816V). Ascites (2200 mL) and non-ruptured esophageal varices with submucosal hemorrhage indicated the presence of severe portal hypertension. Although there was no evidence of liver cirrhosis, the hepatic sinusoids were clogged with tumor cells, with a tendency to be more severe in the perivenular areas, and the lumens of central veins were obliterated by tumor cell infiltration. The present case demonstrates that non-cirrhotic portal hypertension due to blocking of sinusoidal and venous flow could be a serious complication in mast cell leukemia.

show abstract

Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

Mast cell leukemia with rapidly progressing portal hypertension

Yoshida

Nishikawa²,

Yamamoto³

et al. 2009

Pathology International

View full text Add to dashboard Cite

show abstract

“…[1][2][3][4][5][6] Myeloablative allogeneic BMT has been attempted in at least one case of very aggressive MC disease with a novel c-kit mutation; however, the patient died within 4 months after the BMT. 11 In two other cases of systemic mastocytosis with associated MDS/AML treated with allogeneic BMT, the BM and liver mastocytosis persisted despite the complete remission of the associated hematological disease.…”

Section: Discussionmentioning

confidence: 99%

“…All patients with MCL who received chemotherapeutic agents did poorly with an overall survival of less than 1 year, despite transient remissions in some cases. [2][3][4][5][6] Somatic mutations in c-kit appear to be involved in the pathogenesis of mast cell (MC) disorders; however, the c-kit tyrosine kinase inhibitors that are currently available probably have a very limited role in management of the common forms of mastocytosis, which is still under investigation. 7,8 Allogeneic bone marrow transplantation (BMT) may be a curative therapeutic approach in MCL for three reasons: (1) MC originate from a BM hematopoietic CD117 þ /CD34 þ stem cell and demonstrate a number of characteristics that indicate they are myeloid cells.…”

mentioning

confidence: 99%

Evidence for a graft-versus-mast-cell effect after allogeneic bone marrow transplantation

Spyridonidis

Thomas

Bertz

et al. 2004

Bone Marrow Transplant

View full text Add to dashboard Cite

Summary:Mast cell leukemia (MCL) is a rare form of aggressive mastocytosis with a reported median survival below 6 months. Casuistic reports suggest the effectiveness of allogeneic bone marrow transplantation (BMT) for MCL. However, these reports lack clear evidence for a graftversus-mast-cell (GvMC) effect. We prospectively investigated the GvMC at different time points after allogeneic BMT and donor-lymphocyte infusions (DLI). Samples were gathered from a patient with MCL treated with allogeneic BMT from an unrelated HLA identical donor. Parameters for detection of a GvMC effect included flow cytometrical analysis of mast cell (MC) populations in peripheral blood and BM, BM smear and histology, chimerism analysis of flow cytometrically sorted BM CD117 þ /CD34À MC and testing for anti-mast cell reactivity of donor lymphocytes by interferon (IFN)-c ELISPOT. DLIs reduced MC from 5 to 0.5%. MC chimerism analysis demonstrated a complete recipient genotype after BMT, suggesting that the persistent mastocytosis was part of residual neoplastic disease. At 3.7 years after BMT, there is some evidence for relapse. In summary, BMT and DLIs attenuated the mastocytosis from an aggressive to an indolent form and may have improved the patients' prognosis. The in vitro data of our study indicate for the first time the existence of a GvMC effect.

show abstract

“…Normally there is no CD34 expression after the myeloblastic stage [4,5]. In our case, abnormal proliferated mast cells can express CD34 antigen.…”

mentioning

confidence: 91%