open access

Vol 51, No 1 (2020)
Case Report
Published online: 2020-03-01
Submitted: 2019-09-22
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Systemic mastocytosis with chronic myelomonocytic leukemia followed by transformation into acute myeloid leukemia

Marta Panz-Klapuch, Krzysztof Woźniczka, Anna Koclęga, Anna Kopińska, Kinga Boral, Iwona Grygoruk-Wiśniowska, Grzegorz Helbig
DOI: 10.2478/ahp-2020-0011
·
Acta Haematol Pol 2020;51(1):51-55.

open access

Vol 51, No 1 (2020)
Case Report
Published online: 2020-03-01
Submitted: 2019-09-22

Abstract

Introduction

Systemic mastocytosis (SM) with an associated hematological neoplasm (SM-AHN) constitutes about 40% of all patients with SM. AHN commonly includes myeloid neoplasms and chronic myelomonocytic leukemia (CMML) is seen in about 30% of these patients.

Case report

A 67-year-old male presented to hematologist with fatigue and significant weight loss. Abdominal ultrasound and computed tomography (CT) detected hepatosplenomegaly, abdominal lymphadenopathy, and ascites. He was anemic with leukocytosis and eosinophilia. Trephine biopsy showed > 30% of spindle-shaped mast cells. The mutation was present. Serum tryptase level was elevated to 62 ng/mL. The patient was diagnosed with aggressive SM and received six cycles of cladribine with partial response. Three years later, he developed severe anemia. Eosinophilia and monocytosis (5.6 × 10/L) were demonstrated in blood film. Hepatosplenomegaly and abdominal lymphadenopathy were also present. Trephine biopsy did not demonstrate the presence of spindle-shaped mast cells, but dysplasia in erythroid and myeloid lineages was evident. The histological result of lymph node biopsy as well as blood and bone marrow findings were in line with CMML. He received hydroxyurea, but he transformed soon into fatal acute monocytic leukemia.

Conclusions

The prognosis of SM-AHN depends on AHN component. Leukemic transformation of AHN component may occur in a proportion of patients.

Abstract

Introduction

Systemic mastocytosis (SM) with an associated hematological neoplasm (SM-AHN) constitutes about 40% of all patients with SM. AHN commonly includes myeloid neoplasms and chronic myelomonocytic leukemia (CMML) is seen in about 30% of these patients.

Case report

A 67-year-old male presented to hematologist with fatigue and significant weight loss. Abdominal ultrasound and computed tomography (CT) detected hepatosplenomegaly, abdominal lymphadenopathy, and ascites. He was anemic with leukocytosis and eosinophilia. Trephine biopsy showed > 30% of spindle-shaped mast cells. The mutation was present. Serum tryptase level was elevated to 62 ng/mL. The patient was diagnosed with aggressive SM and received six cycles of cladribine with partial response. Three years later, he developed severe anemia. Eosinophilia and monocytosis (5.6 × 10/L) were demonstrated in blood film. Hepatosplenomegaly and abdominal lymphadenopathy were also present. Trephine biopsy did not demonstrate the presence of spindle-shaped mast cells, but dysplasia in erythroid and myeloid lineages was evident. The histological result of lymph node biopsy as well as blood and bone marrow findings were in line with CMML. He received hydroxyurea, but he transformed soon into fatal acute monocytic leukemia.

Conclusions

The prognosis of SM-AHN depends on AHN component. Leukemic transformation of AHN component may occur in a proportion of patients.

Get Citation

Keywords

advanced systemic mastocytosis; systemic mastocytosis with an associated hematological neoplasm; chronic myelomonocytic leukemia; cladribine; Array

About this article
Title

Systemic mastocytosis with chronic myelomonocytic leukemia followed by transformation into acute myeloid leukemia

Journal

Acta Haematologica Polonica

Issue

Vol 51, No 1 (2020)

Pages

51-55

Published online

2020-03-01

DOI

10.2478/ahp-2020-0011

Bibliographic record

Acta Haematol Pol 2020;51(1):51-55.

Keywords

advanced systemic mastocytosis
systemic mastocytosis with an associated hematological neoplasm
chronic myelomonocytic leukemia
cladribine
Array

Authors

Marta Panz-Klapuch
Krzysztof Woźniczka
Anna Koclęga
Anna Kopińska
Kinga Boral
Iwona Grygoruk-Wiśniowska
Grzegorz Helbig

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