Priapism: presentation of rare haematological disease

Abstract

Monoclonal gammopathies are conditions where abnormal
amounts of immunoglobulins are produced by a clone developed
from a single pro-germ cell. In some cases, monoclonal gammopathies may occur as a result of abnormal B cells, which have
not yet developed into plasma cells. This type of gammopathy
is seen in leukaemia or lymphoma. Hypergammaglobulinemia
increases serum viscosity being the most common cause of
hyperviscosity syndrome. Symptoms of hyperviscosity usually
appear when the normal serum viscosity of 1.4 to 1.8 cp reaches
4 to 5 cp, corresponding to a serum immunoglobulin M (IgM) level
of at least 3 g/dL, IgG level of 4 g/dL, and an IgA level of 6 g/dL.
Symptoms of hyperviscosity may include constitutional symptoms,
bleeding and ocular, neurological or cardiovascular manifestations.
Several etiologic factors have been associated with priapism.
Major etiologic categories are hematologic dyscrasias, neurologic
conditions, nonhematologic malignancies, trauma, erectile dysfunction pharmacotherapy, pharmacologic exposure and idiopathic
factors. Long standing priapism due to malignant lymphoma is
a rare incident. We report a case of lymphoma with abnormal
production of serum IgM (more than 5 g/dL) and priapism as first
clinical manifestation.