Recurrent gastrointestinal bleeding associated with myelofibrosis and diffuse intestinal telangiectasias

A TO-year-old retired nurse was admitted in November 1986 with hematnchezia and early satiety followed by recurrent gastrointestinal bleeding. Fifteen years earlier, she had been admitted with abdominal pain, bloody diarrhea, and severe dehydration. At that time, the rectum was normal at sigmoidoscopy; a barium radiograph study of the colon showed ulceration and thumbprinting in the ascending and proximal transverse colon; results of barium studies of the upper gastrointestinal tract and small intestine as well as a visceral arteriogram were normal. A presumptive diagnosis of ischemic colitis was made, and the patient’s acute illness resolved without specific therapy. Ten years before admission, the patient developed symmetric polyarticular arthralgias and a normochromic, normocytic anemia. She began aspirin therapy for presumed seronegative rheumatoid arthritis. One year later she had a month-long episode of fevers and leukopenia (white blood cell count, 3100) with marked leftward shift. A bone marrow aspiration and biopsy were nondiagnostic. Despite extensive evaluation, a specific etiology was not discovered, and the patient’s signs and symptoms resolved spontaneously. Four years before admission, she developed a chronic nonproductive cough. Wheezing was intermittently noted, and an allergist diagnosed “pollen asthma.” Despite weekly inoculations of “mixed allergens” and oral aminophylline, her dry cough persisted. A chest radiograph was interpreted as normal with the exception of calcified granulomas in the upper lobes. Results of pulmonary function tests were otherwise normal. During the z months before admission, she noted progressive fatigue and a 20-lb weight loss associated with early satiety. On admission for hematochezia, her medications included 12 buffered aspirin tablets per day for arthritis, thyroid hormone supplement for hypothyroidism, and hydrochlorothiazide for hypertension. On physical examination she was tachycardic but normotensive. Physical examination was also remarkable for a spleen tip palpated 3 cm below the left costal margin and bright red blood on rectal examination. Laboratory studies showed a hematocrit of 0.23 (23%) with a mean corpuscular volume of 80 fL, a white blood cell count of 5900, and a platelet count of 256,000. The peripheral smear showed microcytic and hypochromic red blood cells with occasional tear drops, acanthocytes, eliptocytes, and nucleated red blood cells; occasional metamyelocytes and myelocytes were present. An elevated partial thromboplastin time (PTT) of 39.3 seconds (control, 29.4 seconds) was attributed to heterozygous factor XI (FXI) deficiency with an FXI level of 38%; prothrombin and bleeding times were normal. A leukocyte alkaline phosphatase score was normal, as were serum electrolyte, blood urea nitrogen, creatinine, aminotransferase, and lactate dehydrogenase levels. Alkaline phosphatase concentration was elevated at 205 U/L (normal, I 120 UiL), and y-glutamyl transferase level was normal. Except for diffusely sclerotic vertabrae and hummeri, a chest radiograph was unremarkable. Management included red blood cell transfusions, raniti-