CASE REPORT
A 62-year-old Caucasian female presented with a tender,
erythematous to violaceous nodular eruption for 4
weeks (Figure 1). She concomitantly reported abdominal
discomfort, lower back pain, fatigue, and anorexia. Laboratory
results upon initial presentation revealed acute kidney injury
and she was admitted for further workup. At that time she
demonstrated marked leukocytosis with blasts, eosinophilia,
basophilia, and increased lactate dehydrogenase. A retroperitoneal
ultrasound revealed retroperitoneal lymphadenopathy
and a diffusely enlarged uterus with fluid collection.
The patient underwent an endometrial biopsy, skin biopsy, and
bone marrow biopsy. A bone marrow aspirate demonstrated
70% cellular marrow, 65% of which was in blast form in a diffuse
interstitial pattern, hypercellularity with large blasts, and
markedly decreased hematopoietic elements. Flow cytometry
of the aspirate showed the atypical cells to be CD38/CD2+ with
interpretation as large cell neoplasm, favoring a plasmacytic
etiology. Skin and endometrial biopsies demonstrated atypical
leukocytes with a plasmacytic origin, which were similar
to those found in the bone marrow aspirate.
Treatment was initiated with bortezomib, doxorubicin, vincristine,
lenalidomide and dexamethasone, and hemodialysis three
times weekly, and although initially promising, the patient deteriorated
and expired about 43 days after initial presentation.
Further cytogenetic testing was not obtained prior to her demise.
Plasma cell leukemia (PCL) is a rare variant of multiple myeloma
(MM), a monoclonal gammopathy, the current pathophysiology
of which is not yet well understood. Our patient presented
atypically with the presence of leukemia cutis, involvement of
endometrium but the absence of bone pain or organomegaly.
PCL may have extramedullary involvement, but cutaneous involvement
as an initial presentation has only been described
very sporadically in the literature.1
Cutaneous involvement of plasma cell dyscrasias typically occurs
after chemotherapy, particularly thalidomide or stem cell
transplantation, and more often in MM with later diagnosis of
plasma cell leukemia rather than the initial presentation.2 The
other described cases of cutaneous involvement of PCL often
describe violaceous to hemorrhagic tender, infiltrated deep-set
papules and nodules. Leukemia cutis is a much more commonly
observed phenomenon in lymphocytic leukemias, which is
a poor prognostic factor with a one-year survival rate of 12%.3
Subcutaneous plasmacytoma growth has been hypothesized to
be a result of transformation of the adhesion molecules LFA-1
and ICAM-1 at some point during disease course, facilitating
plasma cell migration from the bone marrow towards extra-
