Priapism - A Rare Presentation in Chronic Myeloid Leukemia: Case Report and Review of The Literature
Priapism - A Rare Presentation in Chronic Myeloid Leukemia: Case Report and Review of The Literature
Priapism - A Rare Presentation in Chronic Myeloid Leukemia: Case Report and Review of The Literature
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CASE REPORT
A previously healthy 21-year-old man was
referred from local hospital for treatment of priapism
and hyperleukocytosis. His penis remained erect,
From the Department of Emergency Medicine, 1Division of Hematology-Oncology, Chang Gung Memorial Hospital, Taipei.
Received: Mar. 20, 2002; Accepted: Aug. 20, 2002
Address for reprints: Dr. Shy-Shin Chang, Department of Emergency Medicine, Chang Gung Memorial Hospital. 5, Fushing Street,
Gueishan Shiang, Taoyuan, Taiwan 333, R.O.C. Tel.: 886-3-3281200 ext. 2505; Fax: 886-3-3287715; E-mail:
[email protected]
Meng-Wei Chang, et al
Priapism in Chronic Myeloid Leukemia
DISCUSSION
Priapism is an involuntary prolonged erection
unrelated to sexual activity and cannot be relieved by
ejaculation. Most priapism is painful but not all
cases. Priapism is defined as either low-flow
(ischemic) or high-flow (non-ischemic).(4) Low-flow
or ischemic priapism results from pathologically
decreased penile venous outflow that eventuates in
stasis. Intracavernosal blood sampling reveals acidosis and a decrease in oxygen tension. (5) Clinically,
low-flow priapism manifests as a painful, rigid erection. This type is more common and represents an
actual emergency because irreversible cellular damage and fibrosis occur if treatment is not adminis-
289
tered within 24 to 48 hours.(6) It will result in longterm sequela of erectile dysfunction or predisposition
to frequent, prolonged episodes of priapism.(7) The
cause of low-flow priapism including idiopathic,
hematologic disorders,(8-9) tumor infiltrate,(10) or drugs
induced.(11-12) High-flow or arterial priapism differs in
that it results from increased arterial inflow into the
cavernosal sinusoids, which overwhelms venous outflow and clinical presentation was painless. In contrast to low-flow priapism, intracavernosal blood
sampling from patients with high-flow priapism
reveals bright red oxygenated blood,(5) and thus irreversible cellular damage and fibrosis are rare.(6) The
type of priapism is usually due to penis or perineum
trauma that results in injury to the internal pudendal
artery.(13-15) This establishes a fistula between the cavernosal artery and the corpus cavernosum that unregulated inflow occur. It is not an actual emergency in
patients with high-flow priapism, and treatment can
be on an elective basis.
Priapism can occur at any age and two peaks in
age distribution is described.(16) A pediatric peak, 510 years old, is noted owing to sickle cell disease in
black patients. The secondary peak occurs in patients
with active sexual activity age of 20-50 years old.
Idiopathic priapism is the most common (64%) while
approximately 20% are related to hematologic disorders.(1,16) In CML, priapism is an unusual presentation and seldom to encounter. Hyperleukocytosis is
though to be the cause of priapism in patients with
leukemia.(4) Four different mechanism is described:
(1) venous congestion of the corpora cavernosa
resulting from mechanical pressure on the abdominal
veins by the splenomegaly (2) Sludging of leukemic
cells in the corpora cavernosa and the dorsal veins of
penis (3) infiltration of the sacral nerves with
leukemic cells (4) infiltration of the central nerve
system. In our case, significant leukocytosis with
hepatosplenomegaly supports the first mechanism in
the pathogenesis. An important aspect of priapism is
that most physicians will never encounter. The poor
experience will result in delay of treatment and irreversible squeal. So all physicians should understand
that long-term sequela can be avoided with prompt
diagnosis and treatment.
To diagnose the underlying pathophysiology of
priapism, the distinction between low and high flow
priapism is important because their associated treatment and prognosis differ. Differentiating between
290
Meng-Wei Chang, et al
Priapism in Chronic Myeloid Leukemia
cal shunt performed to reduce the priapism. The purpose of surgical procedures is to establish a new
venous outflow and restore normal arterial flow to
the corpora cavernosa. The Urologists should also
involve in the early intervention because of their
familiar with the management and complication of
the priapism.
The importance of prompt diagnosis and treatment of priapism cannot be overemphasized, as there
is definite incidence of impotence following this
condition. One study cited 35% and 60% impotence
rates for patients priapistic for 5 days and 10 days,
respectively. (18) So decompression of the penis
should be done as frequently as possible during the
first 24 hours.(2)
Besides the initial relief of priapism, the further
workup and management of the underlying disease
are more important. In our case, with use of a combined urological therapy and oncological treatment
to priapism, the patient was recovery and had
restored long-term potency. In conclusion, priapism
is an uncommon presentation in CML that all physicians should be aware of the disorders and the need
for early intervention and management.
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Meng-Wei Chang, et al
Priapism in Chronic Myeloid Leukemia
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