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1294 Part X: Malignant Myeloid Diseases Chapter 84: Polycythemia Vera 1295

Figure 84–1. A. Patient with erythromelalgia of
hand and fingers. B. Erythromelalgia of feet and toes
that progressed to necrosis and amputation of the
toes. (Used with permission of Steven Fruchtman, MD,
Allos Therapeutics, Princeton, New Jersey.)

A B

SPECIAL CONSIDERATIONS SPENT PHASE OF POLYCYTHEMIA VERA
Surgery The spent phase of PV, also referred to as post-PV MF, is a frequent
More than 75 percent of patients with uncontrolled PV develop compli- and often terminal complication of the disease. 75,78,107 It is characterized
cations during or after major surgery because both bleeding and throm- by a combination of anemia (non–iron deficiency), progressive increase
boses are common. 82,106 Thus, it is advised to normalize blood counts of splenic size (Fig. 84–2), and marrow fibrosis (Chap. 86). The spent
and blood volume before surgical interventions, which may lower the phase may first be noticed when phlebotomy requirements decrease.
frequency of intraoperative and postoperative complications. Thrombocytosis and granulocytosis (often with immature myeloid
cells) are common. In a minority of cases, thrombocytopenia and gran-
Pregnancy ulocytopenia may occur. Affected individuals are frequently symptom-
Chapter 8 discusses the complications of PV in pregnancy. atic with anemia, bleeding, splenic enlargement with early satiety, and/

A B
Figure 84–2. Patient with spent phase of PV with a massive increase of splenic size (A) and surgically removed spleen (B). (Used with permission of
Steven Fruchtman, MD, Allos Therapeutics, Princeton, New Jersey.)

Kaushansky_chapter 84_p1291-1306.indd 1294 9/21/15 11:10 AM