Page 17 - Journal of Special Operations Medicine - Winter 2016

Secondary Hypertension, Erythrocytosis, and
Unilateral Renal Cystic Disease in a Submariner: A Case Report

Angela S. Forbes, DO; Fred E. Yeo, MD

ABSTRACT
Erythrocytosis, or increased red blood cell mass, may neurological evaluation, were normal except for his
be primary as in the case of polycythemia vera (PV), or blood pressure, which was 145/90mmHg. The patient
secondary due to a variety of causes related to erythro- was prescribed acetaminophen.
poietin (EPO) secretion and hypoxia. Chronic pulmo-
nary disease and certain EPO-secreting tumors should One month later, the patient returned to the clinic
be addressed and excluded early during the course of with worsening headaches. His blood pressure was
evaluation for a patient presenting with increased red 160/90mmHg. Physical examination findings again were
blood cell mass. Inclusion of the JAK2 V617F gene mu- unremarkable. A complete blood cell count was obtained
tation in the recent World Health Organization criteria and revealed a hematocrit of 64%. The levels of serum
for the diagnosis of PV allows for facilitated diagnosis electrolytes, glucose, liver-associated enzymes, serum
and guides therapy. EPO levels can be helpful in diag- creatinine, and lactate dehydrogenase were normal. The
nosis and guiding therapy, but in the case of cystic re- patient’s erythropoietin (EPO) level was within normal
nal diseases, EPO levels are often not elevated, creating limits and measured 10.5mIU/mL. A urinalysis was no-
diagnostic uncertainty. This report describes a case of table for trace microscopic hematuria. Janus kinase 2
symptoms directly attributable to erythrocytosis in the (JAK2 V617F) somatic mutation testing was negative.
setting of negative JAK2 mutation and normal EPO lev- Additional laboratory testing results are listed in Table 1.
els. The subsequent discovery of a large cystic renal kid- Electrocardiography revealed normal sinus rhythm. Find-
ney and PV were the leading diagnostic considerations. ings on a chest radiograph were normal. The patient’s
oxygen saturation was 96% on room air. Computed to-
Keywords: erythrocytosis; unilateral renal cystic disease; mography (CT) imaging of the chest, abdomen, and pel-
polycythemia vera vis revealed normal lungs, liver, spleen, and right kidney;
however, there was marked cystic replacement of the left
kidney (Figure 1). Nuclear scanning with 99m-Tc-MAG3
revealed this renal cystic mass to be nonfunctioning.
Introduction
We describe the case of an active duty Servicemember
who presented with a chief complaint of headache symp- Case Resolution
toms directly attributable to erythrocytosis in the setting The patient was referred for hematology evaluation. He
of negative JAK2 mutation and normal EPO levels. underwent seven weekly phlebotomy treatments and
was treated with a daily 81mg aspirin tablet. Given the
size of the cystic kidney and the nature of the eryth-
Case Presentation
rocytosis, surgical excision was advised. The patient
A 26-year-old black man on active duty in the Navy pre- was taken to the operating room for open left nephrec-
sented with a chief complaint of headaches. The head- tomy. The intraoperative course was complicated by a
aches were described as bitemporal, throbbing, and of left tension pneumothorax, which required placement
increasing intensity over the previous 3 weeks. There of a chest tube. After a brief stay in the intensive care
were no fevers, photophobia, nausea, or light sensitivity. unit for ventilator management and intravenous pain
The patient’s medical history was notable for mild indi- control, the patient recovered uneventfully. Pathologic
gestion and a tonsillectomy 2 years before this presenta- evaluation of the left nephrectomy revealed a 22cm cys-
tion. He was a lifelong nonsmoker and was an enlisted tic mass, completely replacing the kidney parenchyma
submariner. Physical examination findings, including (Figure 2). Upon follow-up 6 months later, the patient’s

1