Sickle cell anemia with fever

119 Pages·2010·2.14 MB·English

Editor
Richard H. Sills,Albany, N.Y.
51 graphs, 2 tables, 2003
Basel •Freiburg (cid:127)Paris (cid:127)London (cid:127)New York (cid:127)
Bangalore (cid:127)Bangkok (cid:127)Singapore (cid:127)Tokyo (cid:127)Sydney
Contents
1 Contributors 26 Newborn screening for Reticuloendothelial Disorders
hemoglobinopathies
2 Preface 46 Lymphadenopathy
M.M. Heeney; R.E. Ware
Z. Hochberg 1. Generalized lymphadenopathy
28 Sickle cell anemia with fever
3 Introduction P. Ancliff; I. Hann
A.S. Al-Seraihy; R.E. Ware
R.H. Sills 48 Lymphadenopathy
30 Management of painful
2. Localized adenopathy
vaso-occlusive episodes in
P. Ancliff; I. Hann
Red Cell Disorders sickle cell disease
50 Splenomegaly
M.M. Heeney; R.E. Ware
4 Initial evaluation of anemia 32 Evaluation and management of P. Ancliff; I. Hann
R.H. Sills; A. Deters
anemia in sickle cell disease
6 Microcytic anemia A.S. Al-Seraihy; R.E. Ware
Coagulation Disorders
R.H. Sills; A. Deters
34 Polycythemia (erythrocytosis)
8 Normocytic anemia A.E. Kulozik; A. Deters 52 Evaluation of a child with
R.H. Sills; A. Deters bleeding or abnormal coagulation
36 Red cell transfusion
10 Macrocytic anemia C. Lawlor; N.L.C. Luban; J.C. Porter; R.H. Sills screening tests
R.H. Sills; A. Deters P. de Alarcon; M.J. Manco-Johnson
12 Pancytopenia 54 Evaluation of a child with
R.H. Sills; A. Deters White Cell Disorders thrombocytopenia
14 Anemia in the neonate M. Cris Johnson; P. de Alarcon
38 Leukocytosis
R.H. Sills; A. Deters 56 Thrombocytopenia in the well
L.A. Boxer
16 Neonatal anemia due to neonate
impaired RBC production 40 Eosinophilia P. Waldron; P. de Alarcon
L.A. Boxer
R.H. Sills; A. Deters 58 Thrombocytopenia in the
18 Hemolytic anemia 42 Neutropenia ill neonate
L.A. Boxer
A. Deters; A.E. Kulozik P. Waldron; P. de Alarcon
20 Hemoglobinuria 44 The child with recurrent infection: 60 Platelet dysfunction
leukocyte dysfunction
A. Deters; A.E. Kulozik K. Dunsmore; P. de Alarcon
L.A. Boxer
22 Presumed iron deficiency anemia 62 Thrombocytosis
which fails to respond to oral iron M. Cris Johnson; P. de Alarcon
R.H. Sills; A. Deters 64 Treatment of bleeding in children
24 Thalassemia with hemophilia
A.E. Kulozik; A. Deters M.A. Leary; R.H. Sills; M.J. Manco-Johnson
66 Evaluation of a child with 80 Assessment of a pelvic mass 96 Recognition and management of
hemophilia who fails infusion M. Weyl Ben Arush; J.M. Pearce superior vena cava syndrome
therapy 82 Assessment of a soft tissue mass S.R. Rheingold; A.T. Meadows
M.J. Manco-Johnson M. Weyl Ben Arush; J.M. Pearce 98 Febrile neutropenia
68 Consumptive coagulopathy 84 Assessment of bone lesions P. Ancliff; I. Hann
A. Deters; A.E. Kulozik M. Weyl Ben Arush; J.M. Pearce 100 Management of biopsy tissue
70 Thrombophilia evaluation in a 86 Initial management of a child with in children with possible
newborn infant with thrombosis a newly diagnosed brain tumor malignancies
M.J. Manco-Johnson S. Bailey B.R. Pawel; P. Russo
72 Thrombophilia evaluation in a 88 Supratentorial brain tumors 102 Diagnosis and management
child with thrombosis S. Bailey of pulmonary infiltrates during
M.J. Manco-Johnson 90 Brain tumors of the posterior fossa, chemotherapy
brain stem and visual pathway P. Langmuir; A.T. Meadows
S.Bailey 104 Monitoring for late effects in
Malignant Disorders 92 Initial management of a child with children with malignancies
74 Assessment of a child with a tumor involving or near the A.T. Meadows; W. Hobbie
suspected leukemia spinal cord 106 Useful normal laboratory values
P. Ancliff; I. Hann S. Bailey R.H. Sills
76 Assessment of a mediastinal mass 94 Recognition and management of
M. Weyl Ben Arush; J.M. Pearce tumor lysis syndrome
78 Assessment of an abdominal mass S. Bailey; R. Skinner 108 Index of Signs and Symptoms
M. Weyl Ben Arush; J.M. Pearce 113 Abbreviations
Library of Congress Cataloging-in-Publication Data Drug Dosage. The authors and the publisher have ex- All rights reserved. No part of this publication may be
Practical algorithms in pediatric hematology and erted every effort to ensure that drug selection and translated into other languages, reproduced or utilized
oncology / editor, Richard H. Sills. dosage set forth in this text are in accord with current in any form or by any means, electronic or mechanical,
p. ; cm. recommendations and practice at the time of publica- including photocopying, recording, microcopying, or
Includes bibliographical references and index. tion. However, in view of ongoing research, changes in by any information storage and retrieval system, with-
ISBN 3–8055–7432–0 (spiral bound: alk. paper) government regulations, and the constant flow of in- out permission in writing from the publisher.
1. Pediatric hematology. 2. Cancer in children. formation relating to drug therapy and drug reactions,
I. Sills, Richard H., 1948– the reader is urged to check the package insert for each
[DNLM: 1. Hematologic Diseases – diagnosis – Child. drug for any change in indications and dosage and for Copyright 2003 by S. Karger AG, P.O. Box,
2. Neoplasms – diagnosis – Child. 3. Decision Trees. added warnings and precautions. This is particularly CH–4009 Basel (Switzerland)
4. Diagnosis, Differential. WS 300 P8947 2003] important when the recommended agent is a new www.karger.com
RJ411.P73 2003 and/or infrequently employed drug. Printed in Switzerland on acid-free paper by
618.92’15–dc21 Rheinhardt Druck, Basel
2002043379 ISBN 3–8055–7432–0
Contributors
Pedro de Alarcon, MD M. Cris Johnson, MD Jennifer M. Pearce, MD
University of Virginia Health System University of Virginia Health System Albany Medical College
Charlottesville, VA, USA Charlottesville, VA, USA Albany, NY, USA
Amal S. Al-Seraihy, MD Andreas E. Kulozik, MD, PhD Joanne C. Porter, MD
Pediatric Sickle Cell Program Department of Pediatric Oncology, Albany Medical College
Duke University Medical Center Hematology and Immunology Albany, NY, USA
Durham, NC, USA University of Heidelberg
Susan R. Rheingold, MD
Heidelberg, Germany
Phil Ancliff, MD
Children’s Hospital
Peter Langmuir, MD
Great Ormond Street Hospital for Children NHS Trust University of Pennsylvania School of Medicine
London, UK Children’s Hospital Philadelphia, PA, USA
University of Pennsylvania Medical School
Simon Bailey, MD Pierre Russo, MD
Philadelphia, PA, USA
Royal Victoria Infirmary Children’s Hospital
Christopher Lawlor, MD
University of Newcastle upon Tyne University of Pennsylvania School of Medicine
Newcastle upon Tyne, UK Children’s National Medical Center Philadelphia, PA, USA
The George Washington University Medical Center
Laurence A. Boxer, MD Richard H. Sills, MD
Washington, DC, USA
C.S. Mott Children’s Hospital Albany Medical College
Margaret A. Leary, MD
University of Michigan Albany, NY, USA
Ann Arbor, MI, USA Albany Medical College
Rod Skinner, MD
Albany, NY, USA
Andrea Deters, MD
Royal Victoria Infirmary
Naomi L.C. Luban, MD
Charité-Virchow Medical Center University of Newcastle upon Tyne
Humboldt University Berlin Children’s National Medical Center Newcastle upon Tyne, UK
Berlin, Germany The George Washington University Medical Center
Peter Waldron, MD
Washington, DC, USA
Kimberly Dunsmore, MD
University of Virginia Health System
Marilyn J. Manco-Johnson, MD
University of Virginia Health System Charlottesville, VA, USA
Charlottesville, VA, USA Mountain States Regional Hemophilia and
Russell E. Ware, MD, PhD
Thrombosis Center
Ian Hann, MD
University of Colorado Health Sciences Center and Pediatric Sickle Cell Program
Great Ormond Street Hospital for Children NHS Trust The Children’s Hospital Duke University Medical Center
London, UK Denver, CO, USA Durham, NC, USA
Matthew M. Heeney, MD Anna T. Meadows, MD Myriam Weyl Ben Arush, MD
1
Pediatric Sickle Cell Program Children’s Hospital Rambam Medical Center
Duke University Medical Center University of Pennsylvania School of Medicine Haifa, Israel
Durham, NC, USA Philadelphia, PA, USA
Wendy Hobbie, PNP Bruce R. Pawel, MD
Children’s Hospital Children’s Hospital
University of Pennsylvania School of Medicine University of Pennsylvania School of Medicine
Philadelphia, PA, USA Philadelphia, PA, USA
Preface
2
The term ‘algorithm’ is derived from Practical Algorithms in Pediatric reader. Twenty-five years after com-
the name of the ninth century Arabic Hematology and Oncology is intend- pleting my pediatric residency, I dis-
mathematician Algawrismi, who also ed as a pragmatic text for use at the cover that Pediatric Hematology-On-
gave his name to ‘algebra’. His ‘algo- patient’s bedside. The experienced cology has become a sophisticated
rismus’ indicated a step-by-step logi- practitioner applies step-by-step logi- specialty with solid scientific back-
cal approach to mathematical prob- cal problem solving for each patient ground of which I know so little. I
lem solving. In reading the final prod- individually. Decision trees prepared would still refer my patients to a spe-
uct, written by some of the finest in advance have the disadvantage of cialist with many of the diagnoses,
pediatric hematologist-oncologists in unacquaintedness with the individual symptoms and signs discussed here.
the world and edited by my friend Dr patient. Yet, for the physician who is But, with the help of this outstanding
Richard Sills, it is obvious that the less experienced with a given prob- book, I would refer them after an edu-
spirit of the algorismus has been uti- lem, a prepared algorithm would pro- cated initial workup, and would be
lized to its best. vide a logical, concise, and cost-effec- better equipped to follow the special-
tive approach prepared by a specialist ist’s management.
who is experienced with the given
problem. In the process of writing this
book, I served as the lay non-specialist Ze'ev Hochberg, MD, DSc
Series Editor
Practical Algorithms in Pediatrics
Professor, Pediatric Endocrinology
Meyer Children’s Hospital
Haifa, Israel
Introduction
Algorithms are practical tools to The algorithms addressing hemato- As with any approach that attempts
help us address diagnostic and thera- logic disorders also concentrate on di- to simplify complex problems, there
peutic problems in a logical, efficient agnosis, but include issues of man- will always be exceptions. Each algo-
and cost-effective fashion. Practical agement of conditions such as sickle rithm must be used in the context of
Algorithms in Pediatric Hematology cell anemia, hemophilia and red blood the individual findings of each patient
and Oncology uses this approach to cell transfusions. under examination and in conjunction
assist the clinician caring for children The format is designed to provide with the current published literature.
with blood disorders and possible as much information as possible. The The clinician must always be aware
malignancies. The book is designed diagnostic algorithms sequentially that any individual patient’s presenta-
for the general practitioner and pedia- move to specific diagnoses, and when tion may be atypical enough, or con-
trician who are not exposed to these space allows, to therapy. To provide a founded by concomitant disorders or
problems on a daily basis as well as better sense of which diagnoses are complications, to render our aproach-
residents and trainees in Pediatrics more likely, very common diagnoses es invalid. In addition, advances in di-
and Pediatric Hematology and Oncol- causing each problem are noted in agnosis and management can render
ogy. bold text, the usually larger number current approaches obsolete.
In addressing oncologic problems, of common diagnoses in standard We hope you will find the book
our goal is to efficiently determine font and rare diagnoses in italics. No helpful in managing the children un-
whether children have malignant or algorithm can contain every possible der your care.
benign disorders, and to establish the diagnosis; many rare diagnoses are
specific diagnosis. Details of specific not included while others may be list-
therapeutic regimens for malignant ed in the algorithm but not the text. Richard H. Sills,MD
disorders are not addressed because Cross-references to other algorithms
they should be determined individual- make the book easier to use. An ap-
ly in consultation with a pediatric on- pendix of age-dependent normal val-
My thanks to all the students,
cologist. Algorithms also address the ues and a convenient list of all abbre-
residents, attending physicians and
management of complications which viations used are also provided.
staff at Albany Medical College
may occur at the time of clinical pres-
3
who graciously took the time to
entation, such as superior vena cava
review and edit the algorithms, and
syndrome, febrile neutropenia, and tu-
to Irene and Sara for their support
mor lysis syndrome as well as an ap-
and love.
proach to recognizing the late effects
of treatment. I dedicate this book to the memory
of my father, Sidney Sills.
Red Cell Disorders R.H. Sills · A. Deters Initial evaluation of anemia
IXn i(cid:1)tial evaluation of anemia (cid:1)
4 WBC – Absolute neutrophil count – Platelets – Blood smear (cid:2)
&WBC 7WBC + 7ANC 7WBC +/or ANC 7Platelets &Platelets (cid:3)
± shift to left (cid:3) 7Platelets Nl WBC/ANC
Blood smear
DCT (cid:11)
(see ‘Thrombocytosis’,
(see ‘Leukocytosis’, p 38) p 62)
Borderline (cid:4) (see ‘Pancytopenia’, Borderline (cid:4) + DCT Microangiopathic Nl WBC, ANC, platelets
Platelets p 12) WBC/ANC changes (cid:10)
Nl platelet
count
(see ‘Consumptive
MCV
coagulopathy’, p 68)
Clinical evidence of acute infection
or autoimmune disease
Decreased Normal Increased
Otherwise well Persistent 7ANC Drug usage (cid:12)
± chronic infections
± failure to thrive
(see ‘Microcytic
anemia’, p 6)
(see ‘Normocytic
anemia’, p 8)
(see ‘Macrocytic
anemia’, p 10)
TEC (cid:5) Shwachman-Diamond Drug induced Acute bacterial Acute or Collagen vascular Evans syndrome(cid:9)
syndrome (cid:13) infection (cid:6) chronic viral disorder (cid:8)
illness (cid:7)
R/O DIC if 7platelets Specific Dx and Rx Possible corticosteroids
(cid:14)(cid:1)–– Outline of the initial steps when evaluat- (cid:14)(cid:4)–– Early in the development of pancytopenia (cid:14)(cid:8)–– SLE and other collagen vascular
ing anemia in children. While some specific some cell lines may fall below the normal disorders can present with these hematologic
diagnoses are discussed here, most will be range before others; however, if one cell line is findings. Specific serologic studies may be
found in the seven other algorithms, which are severely affected, the others are usually ap- indicated.
referred to in this stepwise approach. proaching the lower limits of normal.
(cid:14)(cid:11)–– The direct Coombs test identifies im-
(cid:14)(cid:2)–– The wide availability of electronic cell (cid:14)(cid:5)–– Leukopenia and neutropenia occur in munoglobulin and/or complement on the RBC
counters provides the advantage of having the at least 20% of patients with transient erythro- surface and usually indicates AIHA.
RBC indices, WBC, platelet count and usually blastopenia of childhood. The reticulocyte
ANC obtained automatically with the Hb. With count is usually very low. (cid:14)(cid:9)–– Evan’s syndrome is the combination of
these data, the first step in evaluating anemia ITP and AIHA, although commonly only one of
is to determine whether other cell lines are (cid:14)(cid:13)–– Shwachman-Diamond syndrome is a rare these disorders is apparent at any one time. It
also affected. Make sure that the WBC, ab- autosomal disorder characterized by metaphy- is associated with substantial morbidity and
solute neutrophil count (ANC = % bands + seal dysplasia, exocrine pancreatic insufficien- mortality.
% polymorphonuclear neutrophils ×total WBC) cy, failure to thrive, and neutropenia. Anemia
and platelet count are normal. One-third of the and/or thrombocytopenia may also be noted. (cid:14)(cid:10)–– Microangiopathic changes are due to
children with newly diagnosed leukemia will Neutropenia and anemia are also associated mechanical destruction and include fragment-
have a normal total WBC, but their ANC is usu- with copper deficiency; this is very rare and ed RBCs, schistocytes, irregular spherocytes,
ally reduced. The peripheral smear should be associated with either severe malnutrition or and usually thrombocytopenia.
reviewed to ensure that there are no errors the inadvertent deletion of copper from intra-
with the automated counts, as they do occur. venous nutrition.
The RBC indices, particularly the MCV and Selected reading
RDW, can be extremely helpful in organizing (cid:14)(cid:12)–– A wide variety of drugs cause anemia as
Lee M, Truman JT:Anemia, acute; in
the differential diagnosis. well as neutropenia or thrombocytopenia.
Johnston JM, Windle ML, Bergstrom SK,
Cytotoxic drugs do this most commonly but
Gross S, Arceci RJ (eds): Pediatric Medicine,
(cid:14)(cid:3)–– Leukocytosis and/or thrombocytosis fre- others also do so on an idiosyncratic basis.
Emedicine. com, 2002
quently accompany anemia. Many infections Many of these drugs are used in acute infec-
(http://www.emidicine.com)
and inflammatory disorders cause leukocyto- tions already associated with anemia (such as
sis; a shift to more immature neutrophils trimethoprim/sulfamethoxazole or oxacillin), Smith OP, Hann IM, Chessels TM, Reeves
and/or morphologic changes in neutrophils making it difficult to identify the actual cause of BR, Milla P: Haematologic abnormalities
(toxic granulation, Döhle bodies and vacuoliza- the anemia. in Schwachman-Diamond syndrome.
tion) are often noted, particularly with infec- Br J Haematol 1996;94:279–284.
tion. These same disorders frequently cause (cid:14)(cid:6)–– Acute bacterial infection can result in
Truman JT, Lee M:Anemia, chronic; in
the anemia of acute infection or of chronic anemia with neutropenia and/or thrombo-
Johnston JM, Windle ML, Bergstrom SK,
disease. If blasts are in the peripheral blood, cytopenia. If the patient appears septic and is
Gross S, Arceci RJ(eds): Pediatric Medicine,
leukemia is expected. Thrombocytosis is a very thrombocytopenic, complicating DIC should
Emedicine.com, 2002
nonspecific finding, which in children is almost be considered.
(http://www.emidicine.com)
always reactive and related to infection, any in-
flammatory or neoplastic process, stress, he- (cid:14)(cid:7)–– Acute viral illness is the most common Walters MC, Abelson HT: Interpretation
molysis, blood loss and iron deficiency. Prima- cause of anemia with thrombocytopenia or of the complete blood count.
ry thrombocytosis due to the myeloprolifera- leukopenia. The abnormalities are more likely Pediatr Clin N Am 1996;43:623–637.
tive disorder, called essential thrombocytosis to be mild and are almost always transient. In
Welch JC, Lilleyman JS: Anaemia in
or thrombocythemia, is extremely rare in chil- more chronic infection such as HIV or EBV, the
5 children. Br J Hosp Med 1995;53:387–390.
dren. hematologic findings may persist. Consider
HIV with positive risk factors, other symptoms
and failure to resolve.
Red Cell Disorders R.H. Sills · A. Deters Initial evaluation of anemia