Plasma cell leukemia with
hypercalcemia
A 67 year old woman was admitted with a 20 days history of low
back pain which did not improve despite the use of anti-inflammatory
drugs. Five days before admission, drowsiness, fatigue, muscle
weakness, polydipsia, polyuria and vomiting appeared, along with
fever reaching up to 38 °C, but without chills. Clinical examination
revealed pallor, fever (37.6 °C), mild painful hepatosplenomegaly
and cervical microlymphadenopathy. The patient had a diminished
level of consciousness, was confused and had difficulty concentrating,
but had no focal neurological signs. The hematological tests were
as follows: white blood cell count 12.6x109/L
(neutrophils 24%, lymphocytes 38%, monocytes 7%, metamyelocytes
3%, myelocytes 2%, plasma cells 26%), hemoglobin 8.4 g/dL, hematocrit
26.1% and platelets 68x109/L.
The plasma cells were very large, with a blast-like appearance
and intense cytoplasmic basophilia (fig.1).
However, a small population of typical plasma cells, with a clear
perinuclear halo from Golgi apparatus, was also present. Serum
biochemistry showed raised levels of LDH (990 IU/L), urea (124
mg/dL), creatinine (2.3 mg/dL), uric acid (8.6 mg/dL), calcium
(13.6 mg/dL) and CRP (9.4 IU/L). Serum protein electrophoresis
showed a small monoclonal band with quantitative abnormality.
Immunofixation of gamma globulins revealed the presence of an
IgGκ paraprotein. Radiographs of the axial skeleton revealed an
osteolytic lesion in the third lumbar vertebra. The bone marrow
aspiration showed 75% infiltration by plasma cells (fig.
2). Immunophenotyping of the peripheral blood showed that
the plasma cells were positive for CD38 and for cytoplasmic immunoglobulin.
Chemotherapy along with pamidronate was administered. However,
no remission was achieved, and the patient died of sepsis two
months later.
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