Medical Mystery - The Answer

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correspondence patients with chronic pelvic pain and clinically suspected endometriosis. Obstet Gynecol 1999;93:51-8. 4. Zupi E, Sbracia M, Marconi D, Sorrenti G, Zullo F, Polumba

S. Role of medical therapy in the treatment of endometriosis associated pelvic pain: a randomized controlled study. J Minim Invasive Gynecol 2005;12:Suppl:6.

Medical Mystery: A 71-Year-Old Man with Pancytopenia — The Answer To the Editor: The medical mystery in the October 30 issue1 concerned a 71-year-old man with a 10-day history of dizziness, progressive lethargy, and confusion. A complete blood count showed pancytopenia (white-cell count, 2900 per cubic millimeter [normal range, 4000 to 11,000]; hemoglobin, 5.5 g per deciliter [normal range, 14 to 18]; hematocrit, 14.5% [normal range, 40 to 52]; and platelets, 56,000 per cubic millimeter [normal range, 150,000 to 440,000]) as well as macrocytosis (mean cell volume, 118 μm3 [normal

A

range, 82 to 98], with a red-cell distribution width of 22,300 per cubic millimeter [normal range, 10,500 to 15,500]) and a reticulocyte count of 0.4% (normal range, 1.2 to 3.2). The patient reported occasional alcohol use, and there was no evidence of a toxic ingestion or nutritional deficiencies. He had no known autoimmune disease or endocrinopathies. Additional laboratory work showed a vitamin B12 deficiency (B12, 75 pg per milliliter [normal range, 240 to 900]; folate, 6.2 ng per milliliter [normal range, 2.0 to 20]). His

B

Figure 1. Peripheral-Blood Smear and Bone Marrow Aspirate. The blood smear (Panel A) shows macroovalocytes (arrowheads) and hypersegmented neutrophils (arrow). The bone marrow aspirate (Panel B) shows megaloblastic erythroid precursors (arrowheads) and giant bands (arrow). ICM

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Braza

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Line 4-C SIZE Enon ARTIST: mst H/T H/T FILL Combo december33p9 n engl j med 359;26  www.nejm.org  25, 2008 AUTHOR, PLEASE NOTE: Figure has been redrawn and type has been reset. Downloaded from www.nejm.org on December 25,check 2008carefully. . For personal use only. No other uses without permission. Please

Copyright © 2008 Massachusetts Medical Society. All rights reserved. JOB:

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12-25-08

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peripheral-blood smear showed macroovalocytes (Fig. 1A, arrowheads) and hyper­segmented neutrophils (Fig. 1A, arrow). A bone marrow aspirate, performed to evaluate pancyto­penia, showed megaloblastic erythroid precursors (Fig. 1B, arrowheads) and giant bands (Fig. 1B, arrow). The patient was treated with folic acid, 5 mg daily by mouth for 2 months, and vitamin B12 replacement. A complete blood count obtained 7 months later showed a normalization of the red-cell count indexes. Pernicious anemia is suspected but has not been confirmed. Julia M. Braza, M.D., M.S. Parul Bhargava, M.D. Beth Israel Deaconess Medical Center Boston, MA 02215 1. Braza JM, Bhargava P. A medical mystery — a 71-year-old

of

m e dic i n e

Editor’s note: We received 1520 responses for this medical mystery, from 86 countries. Sixtyfive percent of respondents were practicing physicians, 20% were physicians in training, 11% were medical students, and the remaining 4% were other readers. Overall, 77% of respondents identified some type of megaloblastic anemia, and two thirds of this group specifically suggest­ ed a diagnosis of vitamin B12 deficiency or pernicious anemia. The remaining 23% of respondents suggested other diagnoses: 17% suggested an alternative hematologic process, such as an acute or chronic leukemia, multiple myeloma, Waldenström’s macroglobulinemia, or polycythemia vera, and 6% suggested other conditions, including babesiosis, malaria, parvovirus B19, leishmaniasis, or poisoning with lead or arsenic.

man with pancytopenia. N Engl J Med 2008;359:1941.

More on Propranolol for Hemangiomas of Infancy To the Editor: The response of infantile hemangiomas to propranolol reported in the letter by Léauté-Labrèze et al. (June 12 issue)1 catapulted the use of this treatment to first-line status among physicians managing this disease. Not included in their letter was a discussion about initiating and monitoring propranolol use, or about potential risks, which may be unique among these patients. The most common serious adverse effects are bradycardia and hypotension. Infants with very large hemangiomas or miliary hemangiomatosis are at risk for high-output cardiac compromise.2 Propranolol may mask the clinical signs of early cardiac failure and diminish cardiac performance. Propranolol may also blunt the clinical features of hypoglycemia. Sustained hypoglycemia in infancy has been associated with longterm neurologic sequelae.3 We know of two infants who had unrecognized side effects from propranolol. We developed a treatment protocol to optimize safety: baseline echocardiography and 48-hour hospitalization or home nursing visits to monitor vital signs and blood glucose levels. Medica-

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tion is given every 8 hours, with an initial dose of 0.16 mg per kilogram of body weight. If the vital signs and glucose levels remain normal, the dose is incrementally doubled to a maximum of 0.67 mg per kilogram (to a maximum daily dose of 2.0 mg per kilogram). Propranolol should be gradually tapered over a period of 2 weeks. Elaine C. Siegfried, M.D. William J. Keenan, M.D. Saadeh Al-Jureidini, M.D. Saint Louis University School of Medicine St. Louis, MO 63104 [email protected] 1. Léauté-Labrèze C, Dumas de la Roque E, Hubiche T, Bora-

levi F, Thambo J-B, Taïeb A. Propranolol for severe hemangiomas of infancy. N Engl J Med 2008;358:2649-51. 2. Gottschling S, Schneider G, Meyer S, Reinhard H, DillMueller D, Graf N. Two infants with life-threatening diffuse neonatal hemangiomatosis treated with cyclophosphamide. Pediatr Blood Cancer 2006;46:239-42. 3. Burns CM, Rutherford MA, Boardman JP, Cowan FM. Patterns of cerebral injury and neurodevelopmental outcomes after symptomatic neonatal hypoglycemia. Pediatrics 2008;122:65-74.

The Authors Reply: After more than 40 years of clinical use in infants, there is no documented

n engl j med 359;26  www.nejm.org  december 25, 2008

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