INTRODUCTION

Leukemia cutis (LC) is a rare extramedullary manifestation of leukemia usually associated with an advanced course of the disease. It is exceptionally seen in acute lymphoblastic leukemia (ALL), representing only 1–3% of all cases [1–3]. This was the first case ever reported of LC due to pre-B ALL secondary to cytotoxic therapy manifested clinically as an ulcerative lesion.

CASE REPORT

An eighteen-year-old female was referred to us for the presenting involvement of the left labia majora, with swelling, induration, and the presence of a 1.5 x 1.5 cm ulcer with well-defined regular borders and the presence of fibrin in 80% of the wound bed (Fig. 1). The ulcer was painless and had been present for two weeks. Her laboratory analyses showed leukocytosis, neutropenia, lymphocytosis, anemia, and thrombocytopenia, and the peripheral blood smear revealed 35% blasts. Her medical history included the diagnosis of Ewing’s sarcoma IV in March 2022. She received treatment with 8 cycles of the VDC/IE regimen and five sessions of radiotherapy, having the last one in November 2022. A biopsy was taken with the clinical suspicion of leukemia infiltration versus Ewing’s sarcoma metastasis. The biopsy revealed infiltration by pre-B ALL (PAX 5+, CD79a +, TDT +) (Figs. 2a – 2d) The biopsy and bone marrow aspirate confirmed the diagnosis. FISH study for translocation 9:22 was negative, and cytogenetic testing revealed a complex karyotype with del (5) (q31q33), and del (17) (p11.2). The patient received an induction with CALGB 10403. However, she presented multiple complications associated with chemotherapy and her underlying disease and died on day 20 after the beginning of induction.

	Figure 1: Ulcerative infiltration of the labia majora.
	Figure 2: (a) Pre-B ALL infiltration on H&E stain. (b) Immunohistochemical stain. PAX 5 positive. (c) Immunohistochemical stain. CD79a positive. (d) Immunohistochemical stain. TDT positive.

DISCUSSION

LC is extremely infrequent, mostly affecting patients with acute myeloid leukemia (10–15%) and chronic lymphocytic leukemia (4–20%). Cutaneous infiltration in patients with ALL is exceptionally rare, representing 1–3% of all cases, most of which are reported as deriving from T-cell lineage ALL [1,2].

There is little information available on the subject, with only 14 cases (Table 1) [4–17] of LC pre-B ALL reported in the literature. Although the clinical findings of LC vary widely, it is most often described as asymptomatic, dome-shaped, erythematous-violaceous papules, nodules or plaques, and less commonly as macules, erythroderma, or ulcers, among others [1–3].

Table 1: Leukemia cutis in pre-B-cell acute lymphoblastic leukemia.

This case was of special interest because, to our knowledge, it was the first case of LC pre-B ALL secondary to chemotherapy that presented as a genital ulcer. Patients with therapy-related ALL (t-ALL) represent 3–9% of all adults ALL. Unlike de novo ALL, patients with t-ALL have a high proportion of poor-risk cytogenetic features (MLL rearrangement), which are associated with lower overall survival compared to patients with de novo ALL [1–3].

CONCLUSION

It is important to be aware that patients with a history of chemotherapy or radiation therapy may develop t-LLA. When there are skin lesions present, it is crucial to distinguish between a secondary (new) neoplasm versus a recurrence of a known primary, as the treatment and prognosis will differ greatly.

Cutaneous manifestations of leukemia may be highly polymorphic and may mimic a great number of dermatological conditions, making the diagnosis of LC challenging; therefore, it is critical to always consider ulcers among the ample range of possible presentations.

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