Symptoms:
(1) pulmonary: cyanosis, progressive dyspnea, tachypnea, hypoxemia
(2) CNS: headache, confusion, altered mental status
(3) cardiac: tachycardia, heart failure, ischemia, cardiac tamponade
(4) fever without a focus of infection
(5) ocular: retinal vein distention, retinal hemorrhages, papilledema, visual blurring
(6) priapism in males
Chest X-ray may or may not show interstitial infiltrates.
A biopsy showing intravascular leukocytic thrombi occluding or distending small blood vessels is diagnostic.
Type of leukemia and white blood cell count:
(1) AML: risk very high with counts > 200,000 per µL, but it has been reported to occur with counts < 50,000 per µL
(2) ALL: occurs with counts > 100,000 per µL, with greater risk at counts > 200,000 per µL
(3) CML or CMML: occurs with counts > 150,000 per µL
(4) CLL: rare, but may occur with counts > 800,000 per µL
The workup for infectious agents is negative.
Symptoms tend to improve with reduction in the white cell count by leukopheresis and/or chemotherapy but some patients will progress and die.
