Editor Volume 166, Number 1 • January/February 2014 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 FEATURED ARTICLES FRED A. LOPEZ, MD EDITORIAL BOARD Zhuang Feng, MD, PhD 2 Blastic Plasmacytoid Dendritic Cell Neoplasm: Report of a Case MURTUZA J. ALI, MD Jun Zhou, MD Presenting With Lung and Central Nervous System Involvement and RONALD AMEDEE, MD Gail Bentley, MD Review of the Literature SAMUEL ANDREWS, II, MD BOB BATSON, MD Genevieve F. Maronge, MD 10 The Supply of Hematolgoy/Oncology Specialists EDWIN BECKMAN, MD Paragi Gururaja Ramnaryan, MD, MPH GERALD S. BERENSON, MD Perry G. Rigby, MD C. LYNN BESCH, MD JOHN BOLTON, MD Michael H. Moses, MD 15 Treatment of Submucous Cleft Palate With Selective Use of the MICHELLE BOURQUE, JD Mark W. Stalder, MD Furlow Z-Palatoplasty JAMES N. BRAWNER, III, MD David T. Pointer Jr., BS BRETT CASCIO, MD Ryan Wong, MD QUYEN CHU, MD Charles L. Dupin, MD GUSTAVO A. COLON, MD Hugo St. Hilaire, MD, DDS RICHARD COULON, MD LOUIS CUCINOTTA, MD Marc Manix, MD 21 Distal Ventriculoperitoneal Shunt Catheter Migration to the VINCENT A. CULOTTA, JR., MD Anthony Sin, MD Right Ventricle of the Heart - A Case Report JOSEPH DALOVISIO, MD Anil Nanda, MD, MPH, FACS NINA DHURANDHAR, MD JAMES DIAZ, MD, MPH & TM, DR. PH Brian Revis, MD 26 Malposition of a Hemodialysis Catheter in the Accessory Hemiazygos JOHN ENGLAND, MD Mohammad Kazem Fallahzadeh, MD Vein JULIO FIGUEROA, MD Neeraj Singh, MD ELIZABETH FONTHAM, MPH, DR. PH EDWARD FOULKS, MD Lauren E. Richey, MD, MPH 28 Rapid HIV Testing in a New Orleans Emergency Department is HENRY G. HANLEY, MD Elizabeth J. Carpenter, MD Effective in Identifying New HIV Diagnoses and in Linking Patients to ELIAS B. HANNA, MD James M. Barbeau, MD, JD Care LYNN H. HARRISON, JR., MD Christiane M. Hadi, MD, MPH ROBERT HEWITT, MD MICHAEL HILL, MD D. Luke Glancy, MD 34 In Memoriam: Edward S. Connolly, MD LARRY HOLLIER, MD JOHN HUNT, MD Sabrina L. Noah 35 Protecting the Private Practice of Medicine BERNARD JAFFE, MD NEERAJ JAIN, MD TRENTON L. JAMES, II, MD KEVIN KRANE, MD DEPARTMENTS MAUREEN LICHTVELD, MD, MPH FRED A. LOPEZ, MD D. Luke Glancy, MD 36 ECG OF THE MONTH F. BROBSON LUTZ, JR., MD William P. Abide Jr., MD Amaurosis Fugax in a 45-Year-Old Woman DAVID MARTIN, MD JORGE A. MARTINEZ, MD, JD Chris Stark, BS 38 RADIOLOGY OF THE MONTH ELIZABETH MCBURNEY, MD Jagan D. Gupta, MD Progressive Slurring of Speech and Difficulty Reading in a ELLEN MCLEAN, MD Tracy Austin, MD 62-Year-Old Male NORMAN E. MCSWAIN, JR., MD Enrique Palacios, MD REINHOLD MUNKER, MD Harold Neitzschman, MD DAVID MUSHATT, MD JOSEPH NADELL, MD Faisal Musa, MD 41 CLINICAL CASE OF THE MONTH HAROLD R. NEITZSCHMAN, MD Jorge A. Martinez, MD, JD Altered Mental Status and Headache in a Young Man STEVE NELSON, MD Catherine Hebert, MD NORA OATES, MD Matthew Safley, DO DONALD PALMISANO, MD, JD, FACS David Smith, MD PATRICK W. PEAVY, MD Fred Lopez, MD ROBERTO QUINTAL, MD RAOULT RATARD, MD, MS, MPH & TM Theresa Nuttli, MD 46 PATHOLOGY IMAGE OF THE MONTH ROBERT RICHARDS, MD Robin R. McGoey, MD Altered Mental Status, Alcohol Abuse, and Hyperammonemia DONALD RICHARDSON, MD FRANK A. RIDDICK, JR., MD WILLIAM C. ROBERTS, MD DONNA RYAN, MD JERRY ST. PIERRE, MD CHARLES SANDERS, MD OLIVER SARTOR, MD CHARLES SCHER, MD RICHARD SPECTOR, MD JACK P. STRONG, MD PRAMILLA N. SUBRAMANIAM, MD KEITH VAN METER, MD DIANA VEILLON, MD HECTOR VENTURA, MD CHRIS WINTERS, MD GAZI B. ZIBARI, MD Journal of the Louisiana State Medical Society Blastic Plasmacytoid Dendritic Cell Neoplasm: Report of a Case Presenting With Lung and Central Nervous System Involvement and Review of the Literature Zhuang Feng, MD, PhD; Jun Zhou, MD; Gail Bentley, MD Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematopoietic malignancy with almost invariably cutaneous involvement and poor prognosis. We report a case of BPDCN in a 58-year-old man who presented with skin, lymph node, bone marrow, peripheral blood, lung, and central nervous system involvement. To the best of our knowledge, central nervous system (CNS) involvement as initial presenta- tion has not been reported since the latest World Health Organization (WHO) classification of tumors of the hematopoietic and lymphoid tissues in 2008. Review of the literature was performed on BPDCN cases published in 2008-2013 in PubMed. The major clinical, histopathologic, immunophenotypic, and cytogenetic aspects of the disease were discussed. Dermatologists and dermatopathologists should be aware of this rare disease for which nearly half of the patients present with only cutaneous lesions at diagnosis, as it may allow for early diagnosis and appropriate treatment. INTRODUCTION WHO classification renamed it as BPDCN as a distinct entity under the category of myeloid neoplasm.4 The etiology of Blastic plasmacytoid dendritic cell neoplasm (BPDCN) BPDCN is not, but it has been suggested to be associated is a rare hematopoietic malignancy. The recognition and with acute myeloid/myelomonocytic leukemia (Petrella et nomenclature of BPDCN has been dramatically evolving. al., 2005; Herling et al., 2007). BPDCN was first described by Adachi et al. in 1994 as a The literature of formerly called blastic NK-cell lym- CD4+CD56+ cutaneous lymphoma. The World Health Or- phoma published prior to 2008 may be heterogeneous ganization (WHO) classification of tumors of hematopoietic because CD56 can be expressed in other hematopoietic and lymphoid tissue in 2001 named it as blastic natural killer lineages, including true NK lymphoma and acute myeloid (NK)-cell lymphoma due to its CD56 expression.1 Later, leukemia with monocytic differentiation. In addition, the European Organisation for Research and Treatment markers recently developed for PDCs, including CD123 of Cancer (EORTC) classification of cutaneous lymphoma (the interleukin-3 receptor), blood dendritic cell antigen 2 renamed it as CD4+CD56+ hematodermic neoplasm in (BDCA2/CD303),5 and T-cell leukemia/lymphoma 1 (TCL1) 2005.2 BPDCN was first suggested of dendritic cell origin and CD2-associated protein (CD2AP),6 were rarely tested by Lucio et al. in 1999. More recently, it was confirmed as in the literature published prior to 2008. In this article, we a tumor of precursor plasmacytoid dendritic cells (PDCs) report a case of BDPCN presenting with skin, lymph node, (Chaperot et al., 2001; Chaperot et al., 2004). PDCs produce bone marrow, peripheral blood, lung, and central nervous type 1 interferon (IFN1) and play an important role in the system involvement and have reviewed the literature since modulation of innate and adaptive immunity. PDCs are the latest WHO classification of tumors of hematopoietic produced in the bone marrow and account for less than and lymphoid tissue in 2008 to delineate the characteristics 0.1% peripheral blood mononuclear cells. When immune of the disease. response is activated, PDCs can be recruited into lymph nodes, tonsils, spleen, and mucosa-associated lymphoid METHODS tissue.3 PDCs generally are not identified in skin and sub- cutaneous tissue, and how cutaneous involvement almost Light microscopy slides were prepared from paraffin- invariably occurs in BPDCN is unclear. In 2008, the latest embedded tissue sections and stained with routine hema- 2 J La State Med Soc VOL 166 January/February 2014 Figure 1: (A) Bone marrow core biopsy showing a hypercellular marrow with diffuse infiltrate of tumor cells (H&E 400x). (B) Bone marrow aspirate smear showing infiltrate of tumor cells with high nuclear cytoplasmic ratio, irregular nuclei, finely dispersed chromatin, prominent nucleoli, grey-blue agranular cytoplasm with a clear intracytoplasmic vacuole and pseudopodia-shaped cytoplasmic extension (Wright-Giemsa stain 1000x under oil immersion). toxylin and eosin after fixation in 10% neutral buffered CASE REPORT formalin. Immunohistochemical analyses were performed on formalin-fixed paraffin-embedded tissue sections using A 58-year-old man presented with a two-week history the avidin-biotin-peroxidase method and antibodies to CD3, of generalized weakness, fatigue, and dyspnea on exertion CD4, CD5, CD8, CD10, CD20, CD43, CD45, CD56, CD57, in December 2009. On examination, the patient had a 5 cm CD68, and terminal deoxynucleotidyl transferase (TDT). soft, non-tender, purplish-red nodular skin lesion on the CD123 was performed by Mayo Clinic. Three-color flow right forearm that grew larger over the past couple of weeks. cytometric analysis (FACScan, Becton Dickinson, Mountain There were also multiple small purplish papular lesions on View, CA) was performed on fresh cell suspension and the face, back, and left shoulder. Bilateral cervical multiple cerebrospinal fluid (CSF) using antibodies to CD2, CD3, enlarged lymph nodes were present. Upon admission, he CD4, CD5, CD7, CD13, CD16, CD19, CD56, CD57, and TDT. had anemia and thrombocytopenia with white blood cell In situ hybridization for Epstein-Barr virus (EBV), encoded 5,400/ul, hemoglobin 10.1 g/dl, and platelets 84,000/ul. small mRNAs (EBER) were performed. Cytogenetic studies Bone marrow core biopsy showed a markedly hypercellular were performed with standard protocols. Q banding was marrow that was diffusely replaced by medium-sized blasts used for chromosome identification and karyotypes were with high nuclear cytoplasmic (N:C) ratio, scant pale cyto- defined according to the International System for Human plasm, irregular nuclear contour, and prominent nucleoli Cytogenetic Nomenclature. Fluorescence in situ hybridiza- occupying more than 90% of marrow space (Figure 1A). tion (FISH) analysis was performed using LSI TCR alpha/ Few mitotic figures were also noted. Bone marrow aspirate delta dual color break-apart DNA probe (Vysis, Downers smears showed a predominance of blasts with high N:C Grove, IL).