Ann Dermatol.  2019 Dec;31(6):673-677. 10.5021/ad.2019.31.6.673.

Leukemia Cutis in Chronic Neutrophilic Leukemia Associated with Colony Stimulating Factor 3 Receptor Mutation: Clinical Severity Paralleled with Hematologic Abnormality

• Kwon O
• Park J
• Chung H
• Park KD

• KMID: 2461992
• DOI: http://doi.org/10.5021/ad.2019.31.6.673

Abstract

Cutaneous lesions of leukemia cutis (LC) by chronic neutrophilic leukemia (CNL) have been merely reported due to the rare occurrences of CNL. Furthermore cutaneous lesions in relation to clinical severity have been far less studied. A 70-year-old man presented with multiple violaceous papules and excoriations on both lower extremities. The diagnosis was LC based on histologic and laboratory evaluation and the origin was elaborated as CNL with the confirmation of colony stimulating factor 3 receptor (CSF3R) mutation. Interestingly, the patient presented clinical severity in a parallel manner to the hematologic abnormality. To the best of our knowledge, there has been no reported case of CSF3R confirmed LC in CNL featuring explicit skin eruption in relation to laboratory findings.

Keyword

Colony-stimulating factors 3 receptor; Leukemia cutis; Leukemia, neutrophilic, chronic

MeSH Terms

Aged
Colony-Stimulating Factors*
Diagnosis
Humans
Leukemia*
Leukemia, Neutrophilic, Chronic*
Lower Extremity
Skin
Colony-Stimulating Factors

Figure

• 

  Fig. 1 Serial clinical images. (A) Skin lesion at initial visit featuring multiple red to violaceous papules and plaques with severe oozing and edema on the feet, leukocyte count (63,500/mm3) (B) Aggravation of skin lesion after dosage reduction of hydroxyurea (hydroxycarbamide 0.5 g) leukocyte count (113,000/mm3) (C) Aggravation of skin lesion despite initial dosage of hydroxyurea (hydroxycarbamide 1 g) leukocyte count (98,150/mm3) (D) Improvement of skin lesion after double of initial hydroxyurea dosage (hydroxycarbamide 2 g) with concomitant improvement of leukocytosis, leukocyte count (22,000/mm3).

• 

  Fig. 2 (A) Peripheral blood smear exhibits dominantly abundant nature of segmented and band form neutrophils with all stages of neutrophil precursors including promyelocytes, myelocyte and metamyelocyte. (B) Bone marrow biopsy reveals hypercellularity of cells and red marrow with dominant number of neutrophilic granulocytes bearing normal maturation. Myelobastic cells are less than 5% on the captured microscopic field. (C) Scanning magnification of the skin biopsy revealed diffuse infiltration of inhomogenous neutrophils with pustular formation (H&E, ×40). (D) Higher magnification reveals variable sizes and shapes of both the nucleus and the cells with characteristics (H&E, ×400). (E) Both immature and mature neutrophilic components are shown with low degree of atypia in size and shapes (H&E, ×400). (F) Positive immunohistochemical staining result to myeloperoxidase stain is observed (MPO, ×200).

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  Fig. 3 Genetic study of the colony stimulating factor 3 receptor through Sanger gene deoxyribonucleic acid (DNA) sequencing chromatogram analysis. (A) Original transcript of the sequenced data (SCLLAB 20170209-310878). (B) Extracted sequence of the abnormality exhibiting pathologic variant at the 1,853th base-sequence, where cytosine was transferred to thymine, leading to subsequent substitution of threonine to isoleucine (red arrow).

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