Baylor University Medical Center Proceedings July 2017 - 334

Mixed phenotypic acute leukemia with leukemia cutis and
neuroleukemiosis
Rona Joseph Poikayil, MD, Geetha Narayanan, MD, DM, Harish Sugathan, MD, and Lali V. Soman, MBBS

Leukemia cutis and
neuroleukemiosis are
two rare extramedullary manifestations of
acute leukemia. We
report a 32-year-old
woman with multiple
skin lesions and painful
peripheral neuropathy.
Bone marrow biopsy Figure 1. (a) Violaceous nodules and plaques on the lower back. (b) Improvement of the skin lesions after induction chemotherapy.
and skin biopsy confirmed the diagnosis of mixed phenotypic acute leukemia. After induction
Laboratory testing revealed a hemoglobin of 13.9 g/dL,
total leukocyte count of 15,600/mm² with 14% abnormal
chemotherapy, she attained marrow remission, her skin lesion resolved
cells, and a platelet count of 331,000/mm². Peripheral blood
completely, and her neurologic symptoms significantly improved.

xtramedullary manifestations are unusual clinical presentations of acute leukemia reported in 20% to 40%
of patients (1). These include myeloid sarcoma, central
nervous system leukemia, testicular involvement, gingival inﬁltration, leukemia cutis, and neuroleukemiosis. Such
manifestations may follow, precede, or occur concomitantly
with the diagnosis of leukemia. We report a patient with mixed
phenotypic acute leukemia who presented with two distinct
concurrent extramedullary manifestations: leukemia cutis and
neuroleukemiosis.
CASE PRESENTATION
A 32-year-old woman presented with painless growing
violaceous plaque over her lower back. Within 3 months,
she developed multiple other lesions. Additionally, she had
paresthesias of the limbs in the form of numbness, pricking,
and burning pain and features of distal muscle weakness. She
presented to our center following a skin biopsy. Multiple welldeﬁned discrete nontender indurated plaques and nodules
were noted on the lower back, anterior abdominal wall, thighs,
and upper limb, with the largest measuring 6 × 6 cm (Figure
1a). Sensation was preserved over the lesions but peripheral
neuropathy was suspected due to weakness of bilateral hand
and foot muscles with wasting and demonstrable distal sensory deﬁcits.
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smear examination showed 12% blasts. Bone marrow aspirate revealed 80% blasts, and >50% were peroxidase positive
(Figure 2a). Immunophenotyping by ﬂow cytometry demonstrated the blasts expressed the myeloid marker anti-MPO
(strongly positive) and B-cell markers CD10, CD19, CD20,
and cyCD79a. The other myeloid markers (CD13, CD33)
and T-cell markers (cyCD3, CD3, CD5) were negative. This
was consistent with a diagnosis of mixed phenotypic acute
leukemia (B/myeloid). The skin biopsy displayed focal inﬁltration by atypical medium to large cells around skin appendages (Figure 2b). The atypical cells were CD20 positive,
MPO positive, CD5 negative, and CD34 negative, compatible with acute leukemia inﬁltrating the skin. Magnetic resonance imaging showed no evidence of brain parenchymal,
orbital, or intraspinal deposits. Nerve conduction ﬁndings
were consistent with peripheral neuropathy. Cerebrospinal
ﬂuid was normal.
The patient was started on chemotherapy with the hyperCVAD regimen (cyclophosphamide, vincristine, doxorubicin,
methotrexate, cytarabine, and dexamethasone). After induction,
she achieved marrow remission, her skin lesions disappeared
From the Department of Medical Oncology, Regional Cancer Centre, Trivandrum,
India.
Corresponding author: Geetha Narayanan, MD, DM, Department of Medical
Oncology, Regional Cancer Centre, Trivandrum 695011, Kerala, India (e-mail:
geenarayanan@yahoo.com).
Proc (Bayl Univ Med Cent) 2017;30(3):334-335

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