Manual of Pediatric Hematology and Oncology

Philip Lanzkowsky

• Publisher: Academic Press
• Year: 2005
• Edition: 5
• eBook ISBN:
  9780123751553
• Topic: Biological Sciences
• Subtopic: Oncology
• Index: Biological Sciences

Given that there have been considerable advances in the treatment and management of oncologic diseases in children, the fifth edition of this successful clinical manual will be entirely updated to incorporate all current protocols and developments.This edition will be a comprehensive book on patient management, replete with algorithms and flow diagrams on diagnosis and management. Its concise and easy-to-read format will enable readers to make accurate diagnoses and permit them to treat patients without having extensive previous hematologic/oncologic experience. A list of normal values at various ages in children, providing an extremely useful reference for patient management is included. - Selected for inclusion in Doody's Core Titles 2013, an essential collection development tool for health sciences libraries - Offers a concise, systematic approach to all pediatric hematologic and oncologic disorders in one reference manual - Easy-to-read format: multiple tables, charts, and flow-diagrams for diagnosis and management of pediatric hematologic and oncologic disorders - Clear presentation by practicing clinicians, who are also academic researchers, of recent developments in molecular genetics, cytogenetics, immunology, transplantation, and biochemistry

Chapter 1

Classification and Diagnosis of Anemia in Children

Anemia can be defined as a reduction in hemoglobin concentration, hematocrit, or number of red blood cells per cubic millimeter. The lower limit of the normal range is set at two standard deviations below the mean for age and sex for the normal population.^*

The first step in diagnosis of anemia is to establish whether the abnormality is isolated to a single cell line (red blood cells only) or whether it is part of a multiple cell line abnormality (red cells, white cells and platelets). Abnormalities of two or three cell lines usually indicate one of the following:

• bone marrow involvement, (e.g., aplastic anemia, leukemia), or

• an immunologic disorder (e.g., connective tissue disease or immunoneutropenia, idiopathic thrombocytopenic purpura [ITP] or immune hemolytic anemia singly or in combination) or

• sequestration of cells (e.g., hypersplenism).

Table 1-1 presents an etiologic classification of anemia and the diagnostic features in each case.

Table 1-1 Etiologic Classification and Major Diagnostic Features of Anemia in Children

The blood smear is very helpful in the diagnosis of anemia. It establishes whether the anemia is hypochromic, microcytic, normocytic, macrocytic or shows spezcific morphologic abnormalities suggestive of red cell membrane disorders (e.g., spherocytes, stomatocytosis or elliptocytosis) or hemoglobinopathies (e.g. sickle cell disease, thalassemia).

The mean corpuscular volume (MCV) confirms the findings on the smear with reference to the red cell size, e.g., microcytic (<70 fl), macrocytic (>85 fl) or normocytic (72–79 fl). Figure 1-1 delineates diagnosis of anemia by examination of the smear and Table 1-2 lists the differential diagnostic considerations based on specific red cell morphologic abnormalities. The mean corpuscular hemoglobin (MCH) and mean corpuscular hemoglobin concentration (MCHC) are calculated values and generally of less diagnostic value. The MCH usually parallels the MCV. The MCHC is a measure of cellular hydration status. A high value (>35 g/dL) is characteristic of spherocytosis and a low value is commonly associated with iron deficiency.

Figure 1-1 An Approach to the Diagnosis of Anemia by Examination of the Blood Smear. ⁺Spurious macrocytosis (high MCV) may be caused by macroagglutinated red cells (e.g., Mycoplasma pneumonia and autoimmune hemolytic anemia).*Increased number of reticulocytes. **On the basis of increased membrane resulting in an increased membrane/volume ratio. Increased membrane results from exchanges between red cell lipids and altered lipid balance in these conditions.

Table 1-2 Specific Red Cell Morphologic Abnormalities

I. Target cells
Increased surface/volume ratio (generally does not effect red cell survival)

Thalassemic syndromes

Hemoglobinopathies

Hb AC or CC

Hb SS, SC, S-Thal

HbE (heterozygote and homozygote)

HbD

Obstructive liver disease

Postsplenectomy or hyposplenic states

Severe iron deficiency

LCAT deficiency: congenital disorder of lecithin/cholesterol acyltransferase deficiency (corneal opacifications, proteinuria, target cells, moderately severe anemia)

Abetalipoproteinemia

II. Spherocytes
Decreased surface/volume ratio, hyperdense (>MCHC)

Hereditary spherocytosis

ABO ...

Table of contents

• Cover image
• Title page
• Table of Contents
• Copyright
• Dedication
• Contributors
• Introduction Reflection on 50 Years of Progress in Pediatric Hematology-Oncology
• Preface to the Fifth Edition
• Preface to the Fourth Edition
• Preface to the Third Edition
• Preface to the Second Edition
• Preface to the First Edition
• Chapter 1. Classification and Diagnosis of Anemia in Children
• Chapter 2. Anemia During the Neonatal Period
• Chapter 3. Iron-Deficiency Anemia
• Chapter 4. Megaloblastic Anemia
• Chapter 5. Hematologic Manifestations of Systemic Illness
• Chapter 6. Bone Marrow Failure
• Chapter 7. Red Cell Membrane and Enzyme Defects
• Chapter 8. Hemoglobinopathies
• Chapter 9. Extracorpuscular Hemolytic Anemia
• Chapter 10. Polycythemia
• Chapter 11. Disorders of White Blood Cells
• Chapter 12. Disorders of Platelets
• Chapter 13. Hemostatic Disorders
• Chapter 14. Thrombotic Disorders
• Chapter 15. Lymphadenopathy and Splenomegaly
• Chapter 16. Lymphoproliferative Disorders, Myelodysplastic Syndromes and Myeloproliferative Disorders
• Chapter 17. Leukemias
• Chapter 18. Histiocytosis Syndromes
• Chapter 19. Hodgkin Lymphoma
• Chapter 20. Non-Hodgkin Lymphoma
• Chapter 21. Central Nervous System Malignancies
• Chapter 22. Neuroblastoma
• Chapter 23. Renal Tumors
• Chapter 24. Rhabdomyosarcoma and Other Soft-Tissue Sarcomas
• Chapter 25. Malignant Bone Tumors
• Chapter 26. Retinoblastoma
• Chapter 27. Germ Cell Tumors
• Chapter 28. Hepatic Tumors
• Chapter 29. Hematopoietic Stem Cell Transplantation
• Chapter 30. Management of Oncologic Emergencies
• Chapter 31. Supportive Care of Patients with Cancer
• Chapter 32. Evaluation, Investigations and Management of Late Effects of Childhood Cancer
• Chapter 33. Psychosocial Aspects of Cancer for Children and their Families
• Appendix 1: Hematological Reference Values
• Appendix 2: Biological Tumor Markers
• Appendix 3: Pharmacologic Properties of Commonly used Anticancer Drugs
• Index