Pulmonary Leukostasis Mimicking Pulmonary Embolism: Case of The Month
Pulmonary Leukostasis Mimicking Pulmonary Embolism: Case of The Month
Pulmonary Leukostasis Mimicking Pulmonary Embolism: Case of The Month
www.elsevier.com/locate/leukres
Abstract
We report a case of a 32-year-old woman who presented with shortness of breath and pleuritic chest pain, and mismatched
perfusion defects on a ventilation–perfusion scan suspicious for pulmonary embolism. However, subsequent data revealed the
diagnosis of acute myelogenous leukemia with hyperleukocytosis and associated pulmonary leukostasis. Unfortunately, the patient
died despite urgent leukopharesis. Autopsy examination revealed extensive infiltration of leukemic cells in all major organs with
no evidence of pulmonary embolism. This case highlights the clinical, radiographic and histologic features of pulmonary
leukostasis, and reminds the clinician that not all ventilation – perfusion mismatching is due to thromboembolic disease. © 2000
Elsevier Science Ltd. All rights reserved.
One of the most devastating complications of acute A 32-year-old woman presented with progressive
leukemia is the development of hyperleukocytosis and shortness of breath and pleuritic chest pain. She had
the leukostasis syndrome. This syndrome involves the been well until 7 days prior to admission when she
occlusion of small blood vessels primarily in the lungs developed a dry cough, shortness or breath, low grade
and brain, but all major organs may be involved. When fevers and general malaise. On the day of admission,
the pulmonary microvasculature is involved, severe res- the patient presented with worsened shortness of
piratory distress and hypoxemia may occur. Such breath, sharp, pleuritic chest pain and lower extremity
symptoms may be associated with a variety of diagno- edema. A chest radiograph was interpreted as normal,
sis’, but when a chest X-ray is relatively clear, the but an arterial blood gas (ABG) on room air showed a
diagnosis of pulmonary embolism (PE) is commonly paO2 of 39 mmHg. A presumptive diagnosis of PE was
entertained. We present the case of a young woman made, intravenous heparin was started and the patient
who complained of such symptoms and was presump- was transferred to our institution.
tively diagnosed as having PE, whereas these symptoms On physical examination, the patient was diaphoretic
were actually her initial manifestation of acute leukemia and in significant respiratory distress. The temperature
with pulmonary leukostasis. was 38.5°C, the blood pressure 140/85, the pulse 130
beats per min and the respirations 30 per min. Auscul-
Abbre6iations: ABG, arterial blood gas; AML, acute myelogenous tation of the lungs revealed crackles at the left base,
leukemia; DVT, deep vein thrombosis; H&E, Hematoxylin and and there was symmetric edema in both lower
Eosin; PE, pulmonary embolism; V/Q, ventilation/perfusion; WBC, extremities.
white blood cell.
* Corresponding author. Tel.: + 1-802-8476973; fax: +1-802-
Arterial blood gases on room air were pH 7.49, pCO2
8476961. 34 mmHg, pO2 42 mmHg. An electrocardiogram
E-mail address: dkaminsk@zoo.uvm.edu (D.A. Kaminsky) showed sinus tachycardia with a right-sided strain pat-
0145-2126/00/$ - see front matter © 2000 Elsevier Science Ltd. All rights reserved.
PII: S 0 1 4 5 - 2 1 2 6 ( 9 9 ) 0 0 1 6 2 - 9
176 D.A. Kaminsky et al. / Leukemia Research 24 (2000) 175–178
tern. A chest radiograph (Fig. 1) showed small lung Fig. 2. Anterior (top) and posterior (bottom) views of the ventilation
volumes, prominent central pulmonary arteries, and a (left) and perfusion (right) lung scans. Mismatched perfusion defects
hazy airspace filling process at the left base. There was are seen in the right upper lobe.
no evidence of deep vein thrombosis (DVT) by ultra-
sound examination of the lower extremities, but a venti- throughout the vessels and parenchyma of the lungs
lation–perfusion (V/Q) scan (Fig. 2) was read as (Figs. 3 and 4), including the areas of infarction. In
high-intermediate probability for PE, with two mis- addition, there were numerous thrombi in small vessels
matched perfusion defects in the right upper lobe, so without signs of infarction (Fig. 5). These thrombi
heparin was continued. appeared to have been formed recently antemortem,
Subsequent laboratory data revealed a white blood because Trichrome stains did not show the signs of
cell (WBC) count of 305 000 per ml, with 84% blast collagen deposition and organization that would be
forms and 12% promyelocytes, consistent with the expected for more subacute disease. Given the lack of
diagnosis of acute myelogenous leukemia (AML). evidence for lower extremity DVT by ultrasound exam-
The hematocrit value was 31% and the platelet count ination, or clot elsewhere by autopsy examination, the
64 000 per ml, and the patient had coagulation
defects consistent with disseminated intravascular coag-
ulation.
A diagnosis of AML, type M5, with probable
pulmonary leukostasis was made, although con-
comitant PE could not be ruled out, and oxygen,
intravenous fluids, heparin, allopurinol and hydrox-
yurea were administered. Urgent leukapheresis
was performed, with a reduction in the WBC count
by 37% to 190 000 per ml. Unfortunately, the next
day, the patient developed acute right-sided hemiparesis
and cardiac arrest characterized by pulseless elec-
trical activity, and she died despite full resuscitative
efforts.
Autopsy examination showed extensive infiltration
by leukemic cells of all organs, including the heart,
lungs, liver, spleen, kidneys, lymph nodes, bone marrow
and brain. Pulmonary infarction with hemorrhage was
noted in both lower lobes, with an associated arterial Fig. 3. Light micrograph of the lung at autopsy, showing extensive
filling of a pulmonary artery with leukemic cells, as well as leukemic
thrombus in a minor arterial branch of the right lower
infiltration of adjacent alveolar capillaries. Hematoxylin and Eosin
lobe. Extensive leukemic infiltration was noted (H&E), 10 ×.
D.A. Kaminsky et al. / Leukemia Research 24 (2000) 175–178 177