A 58-year-old white man was admitted to the hospital with pain and swelling of the left arm suggestive of thrombophlebit

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A 58 Year Old White Man Was Admitted To The Hospital With Pain And Swelling Of The Left Arm Suggestive Of Thrombophlebit 1 (179.36 KiB) Viewed 26 times

A 58 Year Old White Man Was Admitted To The Hospital With Pain And Swelling Of The Left Arm Suggestive Of Thrombophlebit 2 (81.43 KiB) Viewed 26 times

A 58-year-old white man was admitted to the hospital with pain and swelling of the left arm suggestive of thrombophlebitis. He had presented to his physician 2 days earlier with complaints of pounding headaches, blurred vision, tinnitus, and generalized pruritus, especially after bathing. The patient had been treated for gout for the past 2 months. Family history is unremarkable for any hematologic disorders. The patient is a nonsmoker. On physical examination, the patient's face appeared flushed, and the retinal veins were engorged. Several ecchymoses were apparent on the legs. The spleen tip was palpable three fingerbreadths below the costal margin (indicating moderate splenomegaly). No hepatomegaly or lymphadenopathy was observed. A complete blood count revealed the following values: WBC count: 20.3 x 107L; RBC count: 7.53 x 1012/L; Hgb: 18.2 g/dL; Hct: 58.0%; MCV: 77 fL; MCH: 24.2 pg; MCHC: 31.4%; platelet count: 710 x 109/L. The differential count demonstrated 80% segmented neutrophils, 8% band neutrophils, 9% lymphocytes, and 3% monocytes. Red cell morphology was consistent with a microcytic, hypochromic classification. Subsequent investigations were undertaken as part of the diagnostic workup of the erythrocytosis. Determination of the red cell mass (utilizing the 51 Cr dilution method) was performed and found to be 41 ml/kg (normal male is 36 ml/kg or less). The plasma volume was 40 ml/kg. Arterial oxygen saturation was 94%. The serum iron was 30 pg/dL (normal is 50 to 170) and total iron binding capacity (TIBC), 460 pg/dl (normal is 250 to 450). Serum vitamin B12 was 925 pg/mL (normal is 205 to 876), and vitamin B12-binding capacity was 2600 pg/ml (normal is 1000 to 1022). The LAP score was 198, and the uric acid determination was 10.3 mg/dL. A bone marrow examination revealed 95% cellularity, with panhyperplasia and many large megakaryocytes. Iron stores were absent, and the reticulin content was slightly increased. COMMENT Several findings in the history and physical examination suggest a presumptive diagnosis of PV. The nonspecific symptoms of headache and blurred vision are a result of cerebral circulatory disturbances caused by hyperviscosity. Thrombotic episodes, such as the phlebitis recorded in this patient, are vascular manifestations resulting from the thrombocytosis in conjunction with the hyperviscosity and increased blood volume. The facial plethora and engorged retinal veins are findings associated with conjunctival and mucosal blood vessel congestion. Generalized pruritus occurs in 30% of patients with PV and is related to hyperhistaminemia. The lack of cardiac or respiratory abnormalities and the presence of normal arterial saturation are helpful in ruling out secondary erythrocytosis. The splenomegaly noted is a frequent finding in myeloproliferative disorders. The most important clinical findings supportive of PV are the elevation of the Hgb and Hct, increased red cell mass, and normal plasma volume. Further, evidence of trilineage involvement, leukocytosis, and thrombocytosis, in addition to erythrocytosis and bone marrow panhyperplasia, strongly suggests a diagnosis of PV. Abnormal elevation of the vitamin B12 and B12-binding proteins, uric acid, and LAP are consistent with a myeloproliferative process and are helpful in establishing a diagnosis of PV. The low serum iron and absence of iron stores indicate concomitant iron deficiency. In most patients this is attributed to occult gastrointestinal blood loss and defective platelet function. This patient fulfills all the diagnostic criteria for PV set forth by the PVSG. Because this 58-year-old patient has an elevated Hct and platelet count and is symptomatic (thrombophlebitis), both phlebotomy and myelosuppressive therapy were initiated. Colchicine and allopurinol were used to control the gout experienced by this patient. Pruritus was a persistent complaint despite the management of erythrocytosis by phlebotomy and hydroxyurea. Cyproheptadine was prescribed and found to be successful in controlling the pruritus.
1. Weat laboratory parametes listed in this case indicate a microtic, hypochromic proces? 2 Han erythropoietin level were ordered on this patient would the expeded result be normal, increases, or decreased? 3. What is the reason for the splenomegaly in this patient?