A 54-year-old male with chronic myeloproliferative disorder and pulmonary thrombotic arteriopathy

Abstract

A 54-year-old male was admitted to the hospital in November '97 complaining of frequent episodes of epistaxis for 1 year, and jaundice accompanied by cephalea, anorexia, nausea, and postprandial vomiting. The patient denied the use of alcohol and tobacco, and other significant morbid antecedents.On physical examination, the patient was icteric (+/4), eupneic, with edema (+/4) in the lower limbs. Cardiac and pulmonary examinations were within the normal range. On abdominal examination, a huge ascites was detected with collateral circulation of the portal type. The liver was palpated 8cm from the right costal margin in the midclavicular line, and 6 cm from the xiphoid process, with an increased consistency (++/4), smooth surface, and it was tender on palpation. On skin examination, numerous petechiae were observed, mainly on the thorax.The , 1,030 monocytes per mm 3 . Total bilirubin was 2.88mg/dL, direct bilirubin was 1.59mg/dL, and indirect bilirubin was 1.29mg/dL. Serologic reactions for the diagnosis of hepatitis showed a reactive serum for anti-HBs antibodies, for anti-HBc-IgG antibodies, and a nonreactive serum for HBsAg. Serologic investigation for the diagnosis of hepatitis C was negative. The prothrombin time was 22.8s, the prothrombin activity was 26%, and the activated partial thromboplastin time was 75.2s.On abdominal echography, no flow in the hepatic veins was detected. The abdominal computed tomography showed occlusion of the hepatic veins and partial occlusion of the inferior vena cava ( fig. 1).The bone marrow biopsy revealed hypercellularity due to the granulocytic series with a large number of megakaryocytes ( fig. 2).The diagnoses of chronic myeloid leukemia, BuddChiari syndrome, portal hypertension, and hepatocytic failure were established.Chemotherapy with hydroxyurea was initiated, but the patient did not use it on a regular basis. During the evolution, 7 hospitalizations occurred, the 4 th because of high digestive bleeding due to a gastric ulcer, and the remaining either because of hepatocytic failure with encephalopathy or worsening of the portal hypertension findings. In all hospitalizations, the patient always had a huge ascites, requiring repetitive paracentesis. During the follow-up years, no significant variation in the number of blood-formed elements existed, and no significant variation occurred in hepatic function and in bilirubinemia.Despite the increase in abdominal volume, the patient never reported dyspnea, except in the 5 th hospitalization, when the patient had dyspnea, palpitation, and cyanosis. He remained dyspneic for 7 days. Pulmonary examination was within the normal range. An increase in the intensity of the second cardiac sound in the pulmonary area was detected. No change in blood pressure or in heart rate occurred.On chest X-ray, the pleuropulmonary fields and the cardiac silhouette were normal.The electrocardiogram showed sinus rhythm, a heart rate of 75bpm, QRS axis +30°, with a normal duration of the QRS, and a normal duration and amplitude of the P wave.The last...