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Asymptomatic Green-Gray Nodules on the Chest

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A man in his 60s presented for evaluation of asymptomatic skin lesions on the chest that had been stable since onset 4 months prior. His medical history was significant for hyperlipidemia, familial polyposis, and acute myelogenous leukemia (AML) for which he had undergone an allogenic hematopoietic stem cell transplant 13 months earlier. Cutaneous examination revealed green-gray nodules and plaques on the left and right upper chest (Figure 1). Bilateral cervical, axillary, and inguinal lymph node chains were negative for adenopathy. The patient had not attempted measures to alleviate the nodules and denied experiencing exacerbating factors or pain. Biopsies of the skin lesions were performed.

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B. Chloroma

Histopathologic findings included a diffuse mononuclear infiltrate with expression of lysozyme and myeloperoxidase (MPO) (Figure 2). Additional immunohistochemical staining demonstrated expression of CD34 and was negative for CD4, CD56, and CD123. A myeloid neoplasm sequencing panel was performed on the tissue sample and demonstrated mutations in DNMT3A, IDH1, NPM1 (type A), NRAS, and STAG2, identical to findings noted from the patient’s bone marrow biopsy specimen on initial diagnosis. These findings established a diagnosis of chloroma, also known as myeloid sarcoma.

Myeloid sarcoma results from extramedullary leukemic blasts. When occurring in the skin, it is also known as chloroma because of the green hue imparted by diffuse expression of MPO.1 Myeloid sarcoma presents in patients with a history of AML or myelodysplastic syndromes or during the blast phase of chronic myeloid leukemia.2 On rare occasion, skin lesions may be present without prior history of systemic disease or bone marrow involvement.1 The lesions may occur throughout the body, including the skin and soft tissue.1 Studies have reported that up to 9% of patients with AML have at least 1 site of myeloid sarcoma.3 Additionally, there appears to be an association between allogenic hematopoietic stem cell transplant (HCT) and an increased incidence of extramedullary relapse.4 Extramedullary relapse following HCT most commonly occurs within 12 months to 5 years of transplant.5

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Article Information

Corresponding Author: Catherine G. Chung, MD, The Ohio State University Wexner Medical Center, 2050 Kenny Rd, MMT 930, Columbus, OH 43221 (catherine.chung@osumc.edu).

Published Online: May 27, 2020. doi:10.1001/jamadermatol.2020.1313

Conflict of Interest Disclosures: None reported.

Disclaimer: Dr Kaffenberger is an assistant section editor of JAMA Dermatology, but he was not involved in any of the decisions regarding review of the manuscript or its acceptance.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
1.
Bakst  RL , Tallman  MS , Douer  D , Yahalom  J .  How I treat extramedullary acute myeloid leukemia.   Blood. 2011;118(14):3785-3793. doi:10.1182/blood-2011-04-347229 PubMedGoogle ScholarCrossref
2.
Almond  LM , Charalampakis  M , Ford  SJ , Gourevitch  D , Desai  A .  Myeloid sarcoma.   Clin Lymphoma Myeloma Leuk. 2017;17(5):263-267. doi:10.1016/j.clml.2017.02.027 PubMedGoogle ScholarCrossref
3.
Neiman  RS , Barcos  M , Berard  C ,  et al.  Granulocytic sarcoma.   Cancer. 1981;48(6):1426-1437. doi:10.1002/1097-0142(19810915)48:6<1426::AID-CNCR2820480626>3.0.CO;2-G PubMedGoogle ScholarCrossref
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Wilson  CS , Medeiros  LJ .  Extramedullary manifestations of myeloid neoplasms.   Am J Clin Pathol. 2015;144(2):219-239. doi:10.1309/AJCPO58YWIBUBESX PubMedGoogle ScholarCrossref
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Clark  WB , Strickland  SA , Barrett  AJ , Savani  BN .  Extramedullary relapses after allogeneic stem cell transplantation for acute myeloid leukemia and myelodysplastic syndrome.   Haematologica. 2010;95(6):860-863. doi:10.3324/haematol.2010.025890 PubMedGoogle ScholarCrossref
6.
Alexiev  BA , Wang  W , Ning  Y ,  et al.  Myeloid sarcomas.   Diagn Pathol. 2007;2(42):1-8. doi:10.1186/1746-1596-2-42PubMedGoogle Scholar
7.
Cota  C , Vale  E , Viana  I ,  et al.  Cutaneous manifestations of blastic plasmacytoid dendritic cell neoplasm.   Am J Surg Pathol. 2010;34(1):75-87. doi:10.1097/PAS.0b013e3181c5e26b PubMedGoogle ScholarCrossref
8.
Nelson  CA , Stephen  S , Ashchyan  HJ , James  WD , Micheletti  RG , Rosenbach  M .  Neutrophilic dermatoses.   J Am Acad Dermatol. 2018;79(6):987-1006. doi:10.1016/j.jaad.2017.11.064 PubMedGoogle ScholarCrossref
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Requena  L , Kutzner  H , Palmedo  G ,  et al.  Histiocytoid Sweet syndrome.   Arch Dermatol. 2005;141(7):834-842. doi:10.1001/archderm.141.7.834 PubMedGoogle ScholarCrossref
10.
Succaria  F , Radfar  A , Bhawan  J .  Merkel cell carcinoma (primary neuroendocrine carcinoma of skin) mimicking basal cell carcinoma with review of different histopathologic features.   Am J Dermatopathol. 2014;36(2):160-166. doi:10.1097/DAD.0b013e3182a67f6f PubMedGoogle ScholarCrossref
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