Document X89O69ej8YXkgNOe7xzwdv8ZJ

206 GERBER vi $ asbestosis with pleural plaques; 4) tophaceous gout, secondary. Case 4 An 82-year-old Caucasian newspaper reporter was admitted in January 1969 because of a sub trochanteric fracture of the right femur and hy pertension. He had worked as a tile manufac turer for several years until 40 years previously. On several previous admissions following an at tack of gout in 1958 polycythemia vera had been diagnosed. The patient developed thrombosis of several digital arteries in 1962, cerebrovascu lar insufficiency in 1966, epistaxis in January 1967, and upper gastrointestinal bleeding in October 1967. He was treated with phleboto mies and several injections of radioactive phos phorus ("P). In 1964 he underwent segmental resection of the sigmoid for villous adenoma. Physical examination revealed mild hepatosplenomegaly. The hemoglobin was 12 Gm. per 100 ml., hematocrit 43%, leukocyte count 176,000 with 96% neutrophils and occasional hypersegmented cells. The reticulocytes were 2.4%; platelets, 632,000. Other laboratory tests gave essentially normal results except for elevation of blood uric acid (11.2 mg. per 100 ml.). Roent genogram of the chest revealed diffuse pulmo nary emphysema and fibrosis and slight cardiomegaly. Hip-pinning was performed. The patient died suddenly the following day. Autopsy findings. The brain contained sev eral small old cystic infarcts in the white matter and diffuse ischemic nerve cell change. The lungs were grossly emphysematous and showed patchy interstitial fibrosis histologically. Nu merous megakaryocytes were impacted within the pulmonary capillaries. Occasional asbestos bodies were observed in the wet preparation of ground J lung tissue. The parietal pleura of the posterior thoracic wall and diaphragm con tained several large white hyaline plaques. The heart weighed 450 Gm. and the left ven tricle was moderately hypertrophied. The liver weighed 1,400 Gm.; microscopic examination disclosed centrilobular necrosis and a few mega karyocytes within its sinusoids. The spleen weighed 800 Gm. and contained a firm, redbrown, well-circumscribed nodule 4 cm. in di ameter. Histologic examination proved this to be a hamartoma characterized by thick Billroth cords with relatively few trabeculae and no lymphoid follicles. The remaining splenic tis sue contained scattered small foci of extra medullary hematopoiesis composed largely of erythroid and myeloid precursors accompanied J Waring Blender X 30 sec. by a few hyperchromatic megakaryocytes withiitf the sinusoids, fibroblastic proliferation, an(j) enormous numbers of lipid-laden histiocytes in the red pulp. The lymphoid follicles were re-' duced in number and size. Numerous laree collections Of completely mature granulocytes'' were found in some fields. The vertebral bonemarrow was hypercellular with a fat-to-marrovr: ratio of 1:9 and contained myeloid hyperp[asiawith a myeloid-erythroid ratio of 6-8:1 arid; increased numbers of basophilic and eosino-'T philic granulocytes and their precursors. Large" numbers of megakaryocytes, many of which?) were immature and atypical, were found. Ex amination of the blood vessels disclosed sub-' intimal and adventitial fibrosis. Final diagnoses: 1) a myeloproliferativedisorder, manifested predominantly as polycy themia vera with pronouncedJhyperleukocytosis;; 2) pronounced lipoid histiocyoisis of the spleen,' possibly secondary to platelet^ phagocytosis; $)\ hamartoma of the spleen; 4) pulmonary ashes# tosis with pleural plaque formation; 5) multiple ) small old cystic infarcts of the cerebrum; 6)fe moderate left ventricular hypertrophy; 7) seg7,h mental resection of colon for villous adenoma,-.' remote. Case 5 t A 72-year-old Caucasian woman with a twcK!` year history of diabetes mellitus was admitted;: in 1968 for elective removal of a cataract of did? right eye. She had worked from 1924 to 1942. as a pipe coverer in an asbestos plant. On physi- f cal examination mild hcpatosplenomegaly was. found. The patient's serum was extremely vis-jcous with a positive Sia test, total protein 9.-5 ) Gm. per 100 ml., albumin 2.3 Gm. per 100 ml. , Ultracentrifugation of serum protein showed an;) elevation of 19S macroglobulin to 0.7 Gm. per'; 100 ml. (9.3% of total), atypical macroglobulin, class S 9.7 in the amount of 1,9 Gm. per 100';; ml. (25.3% of total), normal 7S globulins, and; reduced albumin. Immunoelectrophoretic anal-., ysis showed a paraprotein in the gamma-A frac-;: tion, decreased gamma-M fraction, and normals gamma-G, beta-lC, alpha-2 globulin, beta lipot protein, and albumin. The hemoglobin was 11^ Gm. per 100 ml., hematocrit 34%, leukocyte;' count 7,500 with 56 neutrophils, 37 IymphdjU cytes, 1 eosinophil, 6 monocytes. Platelet coun|S was 125,000; reticulocytes 1.6%; sedimentation^ rate 4 mm. per hr. Bone marrow examination revealed rouleau formation and atypical lymph.i# cytic and plasmacytoid infiltrate consistent wittf Waldenstrom's macroglobulincmia. Skeletal roentgenographic studies showed diffuse osteo porosis. There were no punched-out lesion#