Chronic myelomonocytic leukemia masquerading as cutaneous indeterminate dendritic cell tumor

Expanding the spectrum of skin lesions in chronic myelomonocytic leukemia

Sanam Loghavi, Jonathan L. Curry, Guillermo Garcia-Manero, Keyur P. Patel, Jie Xu, Joseph D. Khoury, Carlos A. Torres-Cabala, Priyadharsini Nagarajan, Phyu P. Aung, Bernard Gibson, Brandon Goodwin, Brent Kelly, Brinda R. Korivi, L. Jeffrey Medeiros, Victor G. Prieto, Hagop M. Kantarjian, Carlos E. Bueso-Ramos, Michael T. Tetzlaff

Research output: Contribution to journalArticle

6 Citations (Scopus)

Abstract

Chronic myelomonocytic leukemia (CMML) is a hematopoietic stem cell neoplasm exhibiting both myelodysplastic and myeloproliferative features. Cutaneous involvement by CMML is critical to recognize as it typically is a harbinger of disease progression and an increased incidence of transformation to acute myeloid leukemia. Cutaneous lesions of CMML exhibit heterogeneous histopathologic features that can be challenging to recognize as CMML. We describe a 67-year-old man with a 3-year history of CMML who had been managed on single-agent azacitidine with stable disease before developing splenomegaly and acute onset skin lesions. Examination of these skin lesions revealed a dense infiltrate of histiocytic cells morphologically resembling Langerhans type cells (lacking frank histopathologic atypia), and with the immunophenotype of an indeterminate cell histiocytosis (S100+ CD1a+ and Langerin-). Given the history of CMML, next-generation sequencing studies were performed on the skin biopsy. These revealed a KRAS (p.G12R) mutation identical to that seen in the CMML 3years prior, establishing a clonal relationship between the 2 processes. This case expands the spectrum for and underscores the protean nature of cutaneous involvement by CMML and underscores the importance of heightened vigilance when evaluating skin lesions of CMML patients.

Original languageEnglish (US)
JournalJournal of Cutaneous Pathology
DOIs
StateAccepted/In press - 2017

Fingerprint

Leukemia, Myelomonocytic, Chronic
Langerhans Cells
Skin
Neoplasms
Histiocytosis
Azacitidine
Splenomegaly
Hematopoietic Stem Cells
Acute Myeloid Leukemia
Disease Progression
History
Biopsy

Keywords

  • CMML
  • Cutaneous
  • Indeterminate cell
  • KRAS

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Histology
  • Dermatology

Cite this

Chronic myelomonocytic leukemia masquerading as cutaneous indeterminate dendritic cell tumor : Expanding the spectrum of skin lesions in chronic myelomonocytic leukemia. / Loghavi, Sanam; Curry, Jonathan L.; Garcia-Manero, Guillermo; Patel, Keyur P.; Xu, Jie; Khoury, Joseph D.; Torres-Cabala, Carlos A.; Nagarajan, Priyadharsini; Aung, Phyu P.; Gibson, Bernard; Goodwin, Brandon; Kelly, Brent; Korivi, Brinda R.; Medeiros, L. Jeffrey; Prieto, Victor G.; Kantarjian, Hagop M.; Bueso-Ramos, Carlos E.; Tetzlaff, Michael T.

In: Journal of Cutaneous Pathology, 2017.

Research output: Contribution to journalArticle

Loghavi, S, Curry, JL, Garcia-Manero, G, Patel, KP, Xu, J, Khoury, JD, Torres-Cabala, CA, Nagarajan, P, Aung, PP, Gibson, B, Goodwin, B, Kelly, B, Korivi, BR, Medeiros, LJ, Prieto, VG, Kantarjian, HM, Bueso-Ramos, CE & Tetzlaff, MT 2017, 'Chronic myelomonocytic leukemia masquerading as cutaneous indeterminate dendritic cell tumor: Expanding the spectrum of skin lesions in chronic myelomonocytic leukemia', Journal of Cutaneous Pathology. https://doi.org/10.1111/cup.13039
Loghavi, Sanam ; Curry, Jonathan L. ; Garcia-Manero, Guillermo ; Patel, Keyur P. ; Xu, Jie ; Khoury, Joseph D. ; Torres-Cabala, Carlos A. ; Nagarajan, Priyadharsini ; Aung, Phyu P. ; Gibson, Bernard ; Goodwin, Brandon ; Kelly, Brent ; Korivi, Brinda R. ; Medeiros, L. Jeffrey ; Prieto, Victor G. ; Kantarjian, Hagop M. ; Bueso-Ramos, Carlos E. ; Tetzlaff, Michael T. / Chronic myelomonocytic leukemia masquerading as cutaneous indeterminate dendritic cell tumor : Expanding the spectrum of skin lesions in chronic myelomonocytic leukemia. In: Journal of Cutaneous Pathology. 2017.
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abstract = "Chronic myelomonocytic leukemia (CMML) is a hematopoietic stem cell neoplasm exhibiting both myelodysplastic and myeloproliferative features. Cutaneous involvement by CMML is critical to recognize as it typically is a harbinger of disease progression and an increased incidence of transformation to acute myeloid leukemia. Cutaneous lesions of CMML exhibit heterogeneous histopathologic features that can be challenging to recognize as CMML. We describe a 67-year-old man with a 3-year history of CMML who had been managed on single-agent azacitidine with stable disease before developing splenomegaly and acute onset skin lesions. Examination of these skin lesions revealed a dense infiltrate of histiocytic cells morphologically resembling Langerhans type cells (lacking frank histopathologic atypia), and with the immunophenotype of an indeterminate cell histiocytosis (S100+ CD1a+ and Langerin-). Given the history of CMML, next-generation sequencing studies were performed on the skin biopsy. These revealed a KRAS (p.G12R) mutation identical to that seen in the CMML 3years prior, establishing a clonal relationship between the 2 processes. This case expands the spectrum for and underscores the protean nature of cutaneous involvement by CMML and underscores the importance of heightened vigilance when evaluating skin lesions of CMML patients.",
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AU - Curry, Jonathan L.

AU - Garcia-Manero, Guillermo

AU - Patel, Keyur P.

AU - Xu, Jie

AU - Khoury, Joseph D.

AU - Torres-Cabala, Carlos A.

AU - Nagarajan, Priyadharsini

AU - Aung, Phyu P.

AU - Gibson, Bernard

AU - Goodwin, Brandon

AU - Kelly, Brent

AU - Korivi, Brinda R.

AU - Medeiros, L. Jeffrey

AU - Prieto, Victor G.

AU - Kantarjian, Hagop M.

AU - Bueso-Ramos, Carlos E.

AU - Tetzlaff, Michael T.

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N2 - Chronic myelomonocytic leukemia (CMML) is a hematopoietic stem cell neoplasm exhibiting both myelodysplastic and myeloproliferative features. Cutaneous involvement by CMML is critical to recognize as it typically is a harbinger of disease progression and an increased incidence of transformation to acute myeloid leukemia. Cutaneous lesions of CMML exhibit heterogeneous histopathologic features that can be challenging to recognize as CMML. We describe a 67-year-old man with a 3-year history of CMML who had been managed on single-agent azacitidine with stable disease before developing splenomegaly and acute onset skin lesions. Examination of these skin lesions revealed a dense infiltrate of histiocytic cells morphologically resembling Langerhans type cells (lacking frank histopathologic atypia), and with the immunophenotype of an indeterminate cell histiocytosis (S100+ CD1a+ and Langerin-). Given the history of CMML, next-generation sequencing studies were performed on the skin biopsy. These revealed a KRAS (p.G12R) mutation identical to that seen in the CMML 3years prior, establishing a clonal relationship between the 2 processes. This case expands the spectrum for and underscores the protean nature of cutaneous involvement by CMML and underscores the importance of heightened vigilance when evaluating skin lesions of CMML patients.

AB - Chronic myelomonocytic leukemia (CMML) is a hematopoietic stem cell neoplasm exhibiting both myelodysplastic and myeloproliferative features. Cutaneous involvement by CMML is critical to recognize as it typically is a harbinger of disease progression and an increased incidence of transformation to acute myeloid leukemia. Cutaneous lesions of CMML exhibit heterogeneous histopathologic features that can be challenging to recognize as CMML. We describe a 67-year-old man with a 3-year history of CMML who had been managed on single-agent azacitidine with stable disease before developing splenomegaly and acute onset skin lesions. Examination of these skin lesions revealed a dense infiltrate of histiocytic cells morphologically resembling Langerhans type cells (lacking frank histopathologic atypia), and with the immunophenotype of an indeterminate cell histiocytosis (S100+ CD1a+ and Langerin-). Given the history of CMML, next-generation sequencing studies were performed on the skin biopsy. These revealed a KRAS (p.G12R) mutation identical to that seen in the CMML 3years prior, establishing a clonal relationship between the 2 processes. This case expands the spectrum for and underscores the protean nature of cutaneous involvement by CMML and underscores the importance of heightened vigilance when evaluating skin lesions of CMML patients.

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