EDITOR VOLUME 168 NUMBER 1 • JANUARY | FEBRUARY 2016 ESTABLISHED 1844 D. LUKE GLANCY, MD ASSOCIATE EDITOR L.W. JOHNSON, MD BOARD OF TRUSTEES CHAIR, GEOFFREY W. GARRETT, MD VICE CHAIR, K. BARTON FARRIS, MD SECRETARY/TREASURER, RICHARD PADDOCK, MD ANTHONY P. BLALOCK, MD D. LUKE GLANCY, MD LESTER W. JOHNSON, MD FRED A. LOPEZ, MD EDITORIAL BOARD MURTUZA J. ALI, MD RONALD AMEDEE, MD SAMUEL ANDREWS, II, MD FEATURED ARTICLES BOB BATSON, MD OBINNA OKOYE, MD 2 39-YEAR-OLD FEMALE WITH MILD RESPIRATORY EDWIN BECKMAN, MD GERALD S. BERENSON, MD PHILIP J. DAROCA, JR, MD DISTRESS AND HYPOXIA C. LYNN BESCH, MD UPENDRA KAPHLE, MD JOHN BOLTON, MD FAYEZ KHEIR, MD BRIAN BOULMAY, MD MICHELLE BOURQUE, JD DANIEL SALERNO, MD JAMES N. BRAWNER, III, MD BRETT CASCIO, MD QUYEN CHU, MD SHACKELFORD R, DO, PH 6 A SEVEN-YEAR-OLD MALE WITH CIRCULATING WILLIAM PATRICK COLEMAN III, MD DANSARI J, MD RED BLOOD CELLS SHOWING A THERMAL GUSTAVO A. COLON, MD VEILLON D, MD INJURY-LIKE MORPHOLOGY RICHARD COULON, MD LOUIS CUCINOTTA, MD ONG M, MD VINCENT A. CULOTTA, JR., MD JUSINO T, BS JOSEPH DALOVISIO, MD JEROUDI M, MD NINA DHURANDHAR, MD JAMES DIAZ, MD, MPH & TM, DR. PH COTELINGAM J, MD JOHN ENGLAND, MD JULIO FIGUEROA, MD ELIZABETH FONTHAM, MPH, DR. PH MOIZ VORA, MD 8 ABSOLUTE PLATELET REFRACTORINESS EDWARD FOULKS, MD MENCHU ONG, MD ASSOCIATED WITH HLA ANTIBODIES: BEN GUIDER, MD FIRAS AL-DELFI, MD A CASE REPORT HENRY G. HANLEY, MD ELIAS B. HANNA, MD NESTOR DELA CRUZ, MD LYNN H. HARRISON, JR., MD DIANA VEILLON, MD ROBERT HEWITT, MD MICHAEL HILL, MD JAMES COTELINGAM, MD LARRY HOLLIER, MD JOHN HUNT, MD CATHERINE CHAUDOIR, MD 12 ATYPICAL HUS IN AN ADULT SUCCESSFULLY BERNARD JAFFE, MD NEERAJ JAIN, MD MENCHU G. ONG, MD TREATED WITH ECULIZUMAB TRENTON L. JAMES, II, MD XIN GU, MD STEPHEN KANTROW, MD DIANA VEILLON, MD KEVIN KRANE, MD MAUREEN LICHTVELD, MD, MPH JAMES COTELINGAM, MD FRED A. LOPEZ, MD F. BROBSON LUTZ, JR., MD DAVID MARTIN, MD EVANS ROBERTS III, MD 16 THERAPY-RELATED T/MYELOID MIXED PHENO- JORGE A. MARTINEZ, MD, JD MELODY ONCALE, MD TYPE ACUTE LEUKEMIA IN A PATIENT TREATED ELIZABETH MCBURNEY, MD HANA SAFAH, MD WITH CHEMOTHERAPY FOR CUTANEOUS DIF- ELLEN MCLEAN, MD REINHOLD MUNKER, MD JOHN SCHMIEG, MD, PHD FUSE LARGE B CELL LYMPHOMA DAVID MUSHATT, MD JOSEPH NADELL, MD HAROLD R. NEITZSCHMAN, MD ROGER E. KELLEY, MD 21 WEST NILE VIRUS MENINGO-ENCEPHALITIS: STEVE NELSON, MD JOSEPH R. BERGER, MD POSSIBLE SEXUAL TRANSMISSION NORA OATES, MD BRIAN P. KELLEY, BS DONALD PALMISANO, MD, JD, FACS PATRICK W. PEAVY, MD PAUL PERKOWSKI, MD DEPARTMENTAL ARTICLES PETERMAN RIDGE PROSSER, MD GRETCHEN YANDLE, MD 23 CLINICAL ROBERTO QUINTAL, MD RAOULT RATARD, MD, MS, MPH & TM DEREK J. VONDERHAAR, MD PERSISTENT HYPERTENSION IN A YOUNG ROBERT RICHARDS, MD THEEPHA THAYALAKULASINGAM, MD WOMAN: A CLASSIC PRESENTATION OF CONN’S DONALD RICHARDSON, MD WILLIAM C. ROBERTS, MD ARTHUR ZIESKE, MD SYNDROME DONNA RYAN, MD DAVID SMITH, MD JERRY ST. PIERRE, MD LEE S. ENGEL, MD, PHD CHARLES SANDERS, MD OLIVER SARTOR, MD ROBERT RICHARDS, MD CHARLES SCHER, MD FRED A LOPEZ, MD RICHARD SPECTOR, MD LEE STEVENS, MD JACK P. STRONG, MD D. LUKE GLANCY, MD 27 ECG PRAMILLA N. SUBRAMANIAM, MD ALLAN CHEN, MD IRREGULARLY IRREGULAR CARDIAC RHYTHM KEITH VAN METER, MD DIANA VEILLON, MD IN AN 87-YEAR-OLD WOMAN HECTOR VENTURA, MD CHRIS WINTERS, MD EMMA LINDSEY, BA 29 RADIOLOGY GAZI B. ZIBARI, MD BRIAN J. COPELAND, MD A CASE OF IDIOPATHIC BASAL GANGLIA CALCIFICATION AND BRIEF REVIEW OF THE LITERATURE JOURNAL OF THE LOUISIANA STATE MEDICAL SOCIETY 39-Year-Old Female With Mild Respiratory Distress and Hypoxia Obinna Okoye, MD; Philip J. Daroca, Jr, MD; Upendra Kaphle, MD; Fayez Kheir, MD; Daniel Salerno, MD The diagnosis of cryptogenic organizing pneumonia is usually delayed for several weeks due to treatment for presumed infectious pneumonia. We present a case of cryptogenic organizing pneumonia in a 39-year-old female who presented with shortness of breath and cough. She had both rapid clinical and radiological response to treatment with corticosteroids. INTRODUCTION Exclusion of possible etiologies led to the final diagnosis of cryptogenic organizing pneumonia. An endobronchial ultra- Definitive diagnosis of cryptogenic organizing pneumonia is sound-guided FNA performed one month prior to presentation based on a suggestive clinical radiological presentation, the was negative for malignancy or infections. This procedure was demonstration of the characteristic pathological pattern at lung performed after surveillance chest CT had revealed residual histopathology, and exclusion of possible causes.1 It has been in- mediastinal lymphadenopathy. In addition, review of her home cluded in the American Thoracic Society/European Respiratory medications was not suggestive of any potential culprit and she Society International Multidisciplinary Consensus Classification had no history of connective tissue disease. She had also been of the Idiopathic Interstitial Pneumonias.2 The annual incidence off immunosuppressive medications for the past six months. of organizing pneumonia is 1.97/100 000 population (1.10/100 000 for the cryptogenic form and 0.87/100 000 for the second- DISCUSSION ary form) with a mean age of 67 years at the time of diagnosis.3 Cryptogenic organizing pneumonia usually presents with pro- CASE REPORT gressive and nonspecific symptoms such as mild fever, mild dys- pnea, cough, malaise, anorexia, and weight loss. Symptoms that A 39-year-old woman presented to the hospital with a history will raise suspicion for connective tissue diseases are prominent of fever for three days. She had no medical complaints until two arthralgia or myalgia. Physical examination findings may include years ago when she was diagnosed with Hodgkin’s lymphoma. focal or sparse crackles on auscultation. Subacute presentation Six months ago she underwent autologous stem cell transplan- is typical with onset over a period of less than 3 months. Diag- tation. She had been complaining about shortness of breath nosis is usually delayed for six to twelve weeks due to the con- and dry cough for one month. She denied smoking tobacco. sideration of infectious pneumonia as one possible differential.1 Her only home medication was albuterol inhaler as needed. On examination, the patient appeared in mild respiratory distress with oxygen saturation of 85 percent on room air. Her lung ex- amination revealed crackles in mid lung zones bilaterally. She did not have any skin rash. The rest of her physical examination was normal. Blood work revealed white blood count of 3,200/ mm3 (4000-11,000/mm3), serum Beta-D-Glucan level 33pg/ml (<60pg/ml), procalcitonin level 0.11ng/ml (0.0-0.5ng/ml). Her hemoglobin, electrolytes, creatinine and liver enzymes were all normal. Her posteroanterior chest radiograph is shown in Figure 1. High-resolution chest CT scan (axial view) is shown in Figure 2A and Figure 2B. The patient was started on antibiotics with- out any clinical improvement. Flexible bronchoscopy was per- formed as a result. Surgical pathology of transbronchial biopsy FIGURE 1. Chest radio- specimen of right middle lobe showed granulation tissue with- graph demonstrating in distal air spaces suggestive of organizing pneumonia. Stains patchy ill-defined were negative for any bacterial, fungal or viral infection. airspace opacities mainly in the mid lung zones bilaterally. 2 J La State Med Soc VOL 168 JANUARY/FEBRUARY 2016 JOURNAL OF THE LOUISIANA STATE MEDICAL SOCIETY FIGURE 2A. Axial chest CT scans revealing multifocal areas of airspace disease in a FIGURE 2B. peribronchial distribution with some areas demonstrating a denser peripheral consolida- tive pattern. Our patient was started on prednisone 60 mg daily with plan Some serum biomarkers could be useful to exclude infec- to slowly taper the dose over three to six months. Her clinical tious etiologies for respiratory symptoms. A systematic review improvement was rapid and by day five her cough and short- showed that serum procalcitonin level can be used to guide ness of breath were much better and she had no drop in oxy- initiation and duration of antibiotics in acute respiratory infec- gen saturation upon ambulation. She remained asymptomatic tions and was not associated with increased deaths or treatment on follow-up at three and six months. Corticosteroid therapy failure.9 Procalcitonin levels are elevated in bacterial infections, was discontinued at about 3 months. The dose and duration for more so in invasive bacterial infections. With regards serum Be- corticosteroid therapy has not been established and is based on ta-D-Glucan assay, the pooled sensitivity, and specificity for PCP clinical judgment.1, 4 Generally, duration of treatment should be were nine percent (95 percent confidence interval [95 percent six months or longer. Most patients undergo complete remis- CI], 92-98 percent), and 84 percent (95 percent CI, 83-86 percent) sion with corticosteroid therapy but are still at risk of relapse whereas for invasive fungal infections such as aspergillosis and within one to three months when corticosteroids are tapered or candidiasis the values were 80 percent (95 percent CI, 77-82 discontinued.2 percent), and 82 percent (95 percent CI, 81-83 percent).10 In our case both tests were normal. A potential area for future research The procedure of choice to establish the pathologic diagnosis of could be the use of serum biomarkers of infection in the diag- organizing pneumonia is video-assisted thoracoscopic surgery nostic work-up of patients with possible non-infectious etiolo- (VATS).4, 5 This allows for evaluation of the distribution of the le- gies of respiratory compromise. sions and enhances the exclusion of possible causes. Samples of adequate size can be obtained through VATS. However, trans- bronchial biopsy may be sufficient in cases with typical clinical and radiographic features.1, 4 Also, transbronchial biopsy can be done under local anesthesia.