PRV is frequently discovered incidentally when a complete blood count is performed for another reason. When symptoms are present, they are usually nonspecific. Fatigue, headache, and diaphoresis are common. Pruritis, often following a hot shower, is a frequent complaint. Up to 15% of patients may present with a thrombotic episode. Thrombotic cerebrovascular accidents, coronary artery thrombosis, Budd-Chiari syndrome, and pulmonary embolus all occur. Cavernous sinus thrombosis may also occur in untreated or poorly controlled disease. Erythromelalgia is specific to PRV and ET, and it is associated with an elevated platelet count and paradoxical vasodilation. It is characterized by redness, warmth, and a burning pain affecting the digits and responds promptly to aspirin. Gout may be a presenting manifestation of an MPD. There is an increased incidence of peptic ulcer disease in patients with PRV. Iron deficiency may occur and may initially mask the diagnosis. An elevated hematocrit with microcytosis is a clue to this possibility. Physical findings that may be present include a ruddy complexion, conjunctival injection, retinal vein engorgement, and spleen and/or liver enlargement. Splenomegaly is present in two-thirds of patients at presentation.
Apart from the ruddy complexion, specific head and neck manifestations are unusual. Epistaxis in addition to bleeding from other sites may occur in up to 40% of patients. Pyoderma gangrenosum is reported to occur with the MPD. This is a necrotizing skin ulceration that occurs most commonly on the limbs of patients with inflammatory bowel disease. It can also occur on the face. Manifestations of iron deficiency may include glossitis and cheilosis.
ET is also frequently an incidental finding. Once again, symptoms tend to be nonspecific. Bleeding and thrombotic manifestations occur with equal frequency, both being related to abnormal platelet function. In addition, when the platelet count is markedly elevated, acquired von Willebrand's disease may result from excessive binding of von Willebrand's factor to platelets. This results in mucosal bleeding such as purpura or epistaxis. Head and neck findings include transient visual disturbances such as monocular vision loss (amaurosis fugax) and mucosal or dental bleeding. Bleeding may be present in the presence or absence of concurrent von Willebrand's disease.
Chronic idiopathic MF usually presents with marked fatigue associated with anemia and a hypermetabolic state. Low-grade fevers and weight loss are frequent, as are symptoms related to a varying degree of splenomegaly, which may be massive. Hepatomegaly and splenomegaly occur due to extramedullary hematopoiesis.
Head and neck manifestations of MF are often a result of extramedullary hematopoiesis. This can result in tumors at any site. Lymph nodes may also be involved. Sweet's syndrome can occur in chronic idiopathic MF, presenting as cutaneous plaques or nodules at any site (33). This finding also occurs in a number of other hematological disorders, most commonly AML.
CML is diagnosed as an incidental finding in approximately one-third of patients. The most common symptoms are nonspecific and are related to anemia, bleeding, or infection. Early satiety due to splenomegaly is common.
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