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Generalized leukemia cutis as the initial manifestation of precursor B‐cell acute lymphoblastic leukemia in a young male

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Dear Editor, Leukemia cutis occurs in 1% to 3% of acute lymphoblastic leukemia (ALL) cases and is even rarer in precursor B-cell ALL. We report a case of generalized leukemia… Click to show full abstract

Dear Editor, Leukemia cutis occurs in 1% to 3% of acute lymphoblastic leukemia (ALL) cases and is even rarer in precursor B-cell ALL. We report a case of generalized leukemia cutis as the initial presentation of precursor B-cell ALL in a young male. A 20-year-old male presented with a 4-month history of asymptomatic erythematous nodules and plaques all over the body. His vision in both the eyes was obstructed due to upper eyelid involvement. He denied any constitutional symptoms. Physical examination revealed erythematous, firm to hard, ill-defined nodules and plaques varying in size from 3 × 2 to 8 × 5 cm, at places showing grayishbrown crusting, over both upper eyelids (right > left) (Figure 1A), neck, axillae (Figure 1B), trunk, upper and lower limbs (Figure 1C) and scrotum. Bilateral inguinal lymphadenopathy was present, while hepatosplenomegaly was absent. The differential diagnoses considered were primary cutaneous lymphoma and cutaneous metastases. His laboratory parameters showed hemoglobin of 14.2 g/dL; total leukocyte count of 10.6 × 10/L with 52% polymorphs, 26% lymphocytes, 14% eosinophils, 8% monocytes and platelets of 200 × 10/L; and no atypical cells in the peripheral blood smear. Serum lactic acid dehydrogenase was elevated at 842 IU/L. Histopathological examination from one of the skin nodules depicted unremarkable epidermis and dense infiltration of the entire dermis by monotonous population of large atypical cells (Figure 2A). These cells had high nuclear to cytoplasmic ratio, prominent nucleoli, scanty cytoplasm and were infiltrating between the collagen bundles in Indian file pattern (Figure 2B). On immunohistochemistry, these neoplastic cells were positive for CD34, CD68, CD10, CD43 and terminal deoxynucleotidyl transferase (Figure 2C,D), and negative for CD3, CD5, CD20 and myeloperoxidase (MPO). Fine-needle aspiration cytology from inguinal lymph node showed hypercellularity and monomorphic blast cells. Flow cytometry from bone marrow biopsy identified 56% blasts, which were positive for CD79a, CD10, CD20 and HLA-DR, and negative for cMPO, sCD3, CD34 and CD7, suggesting an infiltrate of precursor B-cells. Wholebody contrast-enhanced computed tomography scan showed homogenously enhancing sheet-like soft tissue over forehead with extension into bilateral orbits, nasal cavity and frontal, ethmoid and maxillary sinuses. Multiple homogenously enhancing enlarged lymph nodes were noted in the neck, mediastinum, axillae, mesentery, retroperitoneum and inguinal regions. Cerebrospinal fluid analysis was negative for malignant cells. A final diagnosis of leukemia cutis secondary to precursor B-cell ALL was made. The patient was treated with Modified BerlinFrankfurt-Munster-95 regimen consisting of two courses of induction phase (IA: prednisolone, daunorubicin, vincristine, L-asparaginase and IB: cyclophosphamide, cytarabine, 6-mercaptopurine), followed by protocol M of high-dose methotrexate and 6-mercaptopurine, two courses of reinduction (IIA: dexamethasone, doxorubicin, vincristine, L-asparaginase and IIB: cyclophosphamide, cytarabine, 6-thioguanine) and lastly maintenance phase (weekly methotrexate, daily 6-mercaptopurine, pulse dexamethasone and vincristine once in 3 months, for 24 months). He is in complete remission since 1 year after the discontinuation of chemotherapy. Precursor B-cell ALL is uncommon, accounting for about 2% of all acute leukemias. Although it occurs most frequently in children, it

Keywords: leukemia cutis; leukemia; precursor; precursor cell

Journal Title: Dermatologic Therapy
Year Published: 2020

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