Lymphomatoid papulosis as a harbinger of chronic lymphocytic leukemia

Dear Editor, We report on a patient with a new diagnosis of lymphomatoid papulosis (LyP) who was subsequently diagnosed with chronic lymphocytic leukemia (CLL). LyP is a CD30+ lymphoproliferative disorder characterized by recurrent papulonodular eruptions that appear malignant histologically but usually follow a benign and indolent course [1]. In 9–18% of patients, LyPmay progress to malignancies such as mycosis fungoides, anaplastic large cell lymphoma, and Hodgkin’s disease (Table 1) [2–7]. To date, there has been one report of LyP associated with acute myeloblastic leukemia (AML) [8] and one report associated with small-cell lymphocytic B cell lymphoma [9]. A previously healthy 78-year-old Caucasian woman presented to the dermatology clinic at our institution with a 5month history of intermittent eruptions of a pruritic rash in the inguinal area, back, thighs, and legs. The rash consisted of erythematous, polymorphic papules that increased in size and coalesced into plaques over the lifespan of the lesion (Fig. 1). The skin lesions appeared in crops and spontaneously regressed within 2 months. In the referring physician’s assessment, two separate skin biopsies were performed and found to be inconclusive, demonstrating spongiotic dermatitis with an inflammatory infiltrate in the dermis. The patient received multiple steroid injections for a presumptive diagnosis of vasculitis, resulting in only temporary relief of symptoms. Laboratory work-up at that time was notable for a lymphocytosis to 62 % (reference range, 12–46 %) and an elevated absolute lymphocyte count of 4.3 K/ μL (reference range, 0.7–4.0 K/μL). However, the patient was evaluated by a hematologist, and malignancy was ruled out due to an absolute lymphocyte count less than 15,000 and no evidence of adenopathy, thrombocytopenia, anemia, or organomegaly. Due to recurrence of lesions, the patient was referred to our institution for further evaluation. She reported feeling well and denied fatigue, fever, chills, or weight loss. A third biopsy showed nonspecific findings and negative direct immunofluorescence. Despite initiating therapy with oral prednisone, the lesions continued to recur, and a fourth skin biopsy was obtained. This demonstrated a superficialto mid-dermal mixed inflammatory infiltrate consisting of predominantly lymphocytes and histiocytes, with few neutrophils. The lymphocytes were large and atypical (Fig. 2a). Immunohistochemical stains were performed and stained positively for CD3, CD20, and CD30. The presence of CD3 highlights a predominance of T cells, whereas CD20 highlights a background of B cells. Staining for CD30 highlighted the larger, atypical Reed-Sternberg-like cells intermingled with inflammatory cells, with the background inflammatory cells greatly outnumbering the CD30-positive tumor cells (Fig. 2b). These findings were consistent with type A lymphomatoid papulosis. Methotrexate 5 mg weekly was added to her regimen, and she was referred to the lymphoma clinic for work-up for underlying malignancy. Upon reevaluation, laboratory tests revealed an elevated white blood cell count to 20.8 K/μL, with an absolute lymphocyte count of 12.2 K/μL (reference range, 1.0– 5.1 K/μL). Flow cytometry of peripheral blood revealed expression of T cell antigen 5 and B cell antigens CD19, CD20, and CD23. There was strong intensity staining for CD45, and no significant CD34-positive blast population was identified. These findings were consistent with a monoclonal CD5-positive B cell lymphocytosis and were consistent with CLL, Rai stage 0. Thinking that the skin lesions could be a manifestation of her underlying CLL, the patient was begun on a single agent bendamustine 90 mg/m. C. S. Ahn : C. S. Orscheln :W. W. Huang (*) Department of Dermatology, Wake Forest School of Medicine, 4618 Country Club Road, Winston-Salem, NC 27104, USA e-mail: whuang@wakehealth.edu DOI 10.1007/s00277-014-2064-6 Ann Hematol (2014) 93:1923–1925