The second edition of Pediatric Hematolgy, edited by the text's
original editors, John S. Lilleyman and Ian M. Hann, as well as a new
editor, Victor S. Blanchette, completely updates and expands upon the
first edition (published in 1992). The new edition grew from 15 to 40
chapters, with contributions by many of the most well-known
investigators and clinicians in pediatric hematology in the world.
The textbook will especially be of value to practicing clinicians,
house staff, and students.
The clinical findings, etiology and pathogenesis, laboratory
abnormalities, treatment, and prognosis of various diseases are
presented succinctly, yet comprehensively, within the books
eight sections. These discussions provide physicians with brief
overviews of the diseases and their current management strategies.
In the first section, which focuses on bone marrow failure syndromes,
Freedman and Doyle discuss inherited and acquired myeloid hypoplasias
and aplasias. Ball and Gordon-Smith review Diamond-Blackfan anemia,
acquired red cell aplasias, and congenital dyserythropoietic anemias.
The section ends with an excellent discussion of the myelodysplastic
syndrome and associated conditions written by Chessells.
In section 2, on red cell disorders, Will reviews iron metabolism,
sideroblastic anemias, and iron overload. Recommendations on the
management of iron overload and iron-loading anemias, including
various chelation strategies, are included. Iron deficiency is
discussed by Wharton, while Elder describes the classification and
pathophysiology of the porphyrias, as well as diagnostic studies and
Section 2 continues with a discussion of megaloblastic anemias from a
pediatric perspective; in this chapter, Rosenblatt and Hoffbrand
cover such topics as inborn errors of metabolism, transport, and
utilization of folate and cobalamin. Neonatal anemias with a
nonimmune etiology are well described by Liesner. Alloimmune and
autoimmune hemolytic diseases are reviewed by Sapp and Bussell; their
chapter includes helpful tables on clinical findings and therapeutic
responses. American readers will enjoy the international perspective
provided by Serjeant in the chapter on sickle cell disease.
Other hemoglobin disorders and variants are covered by Kulozik.
Mentzer and Lubin present erythrocyte membrane defects; Layton and
Bellingham write on metabolic defects of the red cell. The last
chapter in this section, on thalassemias, which is authored by
Oliveri and Weatherall, provides information about genetics,
molecular biology, pathophysiology, clinical features, diagnosis, and
a large section on chelating agents.
In the third section on granulocyte and macrophage disorders,
quantitative and qualitative disorders of granulocytes are reviewed
by Stevens. Webb considers histiocytic syndromes and includes a
helpful review of treatment strategies. The pathology and treatment
of acute myeloid leukemia are masterfully discussed by Pui and Behm
and by Lange and Bunin, respectively. This section concludes with a
chapter on adult-type chronic myeloid leukemia, including molecular
diagnostic approaches and biological response modifiers, written by
Roberts and Dokal.
Section 4, which covers platelet disorders, includes chapters on
inherited and congenital thrombocytopenia (by Smith), immune
thrombocytopenia (Imbach), thrombocytosis (Sutor), and qualitative
platelet defects (Hardisty).
In the fifth section on lymphocyte disorders, the discussion on
immunodeficiency and opsonization by Finn and Cant is not only
comprehensive and well-written but also entertaining. (An example:
If its inside me but isnt me; kill it before it
kills me.) Baruckel, Leblanc, and Schaison review the
histopathology and immunotyping of and genetic changes in
lymphoblastic leukemia. An overview chapter on the clinical features
of lymphoblastic leukemia, as well as Berlin, Frankfort, and Munich
(BFM) therapy and its outcome, is presented by Retter and Schrape.
The chapter by Eden on lymphomas reviews the clinical and laboratory
findings of non-Hodgkins lymphoma and Hodgkins disease
and includes a brief summary on treatment and prognosis for limited-
and advanced-stage disease. This chapter may be problematic for
American readers because it includes the REAL classification of
non-Hodgkins lymphoma, which is unknown to many American clinicians.
Section 6, on coagulation disorders, begins with an excellent chapter
on hemophilia A and B by Lusher, which covers prenatal testing,
clinical manifestations, and management. Lillicrap, Dean, and
Blanchette lend their considerable expertise to a discussion of von
Willebrands disease in one chapter and rare congenital bleeding
disorders in a second. A chapter on acquired bleeding disorders and
neonatal hemostatic problems, authored by Chalmers and Gibson, follows.
The last chapter in this section, by Andrew and Brooker, provides a
comprehensive, clinically useful review of thromboembolic
complications and congenital and acquired thrombophilia, as well as
an evidence-based review of treatment strategies and a discussion of
the pediatric anticoagulation program at the Hospital for Sick
Children in Toronto.
The seventh section on supportive care includes chapters on blood
component therapy, complications of transfusions, and management of
infections in patients with bone marrow failure. Hume initially
describes the techniques used to obtain peripheral red blood cells,
platelets, white blood concentrates, and plasma, and the storage and
subsequent use of these products in various disease states and in
newborns. Manno, Luba, and Piscietta start their chapter with
succinct yet adequate comments on the numerous acute complications
that can occur with transfusions.
This is followed by a discussion of delayed reactions, especially
graft-vs-host disease, and the precautions that should be used to
prevent transmission of infections. In the final chapter of this
section, Dr. Hann reviews the etiology and treatment of patients with
neutropenia and comments on the use of granulocyte colony-stimulating
factor (Neupogen) to ameliorate neutropenia.
The eighth section on the hematalogic effects of systemic disease
begins with an excellent but brief review by Lilleyman on the effects
of organ failure (renal, liver, endocrine, lung, connective tissue)
and infections, especially those caused by the Epstein-Barr virus,
parvovirus B16, TORCH (toxoplasmosis, rubella, cytomegalovirus, and
herpes congenital infections) viruses, and protozoa. (Human
immunodeficiency virus [HIV] infection is not mentioned in this
chapter but is covered elsewhere in the text.) This chapter concludes
with a review of malnutrition and the side effects of drugs.
In the next chapter in this section, Reid rightly points out that
once a diagnosis of nonhematopoietic malignancy involving the bone
marrow is made, few studies are done to evaluate its effect on
hematopoiesis or on the morphology of the tumor cells. Thus, the
literature on this subject is rather limited. He describes the
effects of nonhematopoietic cancers on the red blood cells,
platelets, and white blood cells. The involvement of the marrow by
metastatic neuroblastoma, rhabdomyosarcoma, and primitive
neuroectodermal tumor is presented.
In the last chapter in this section, Dr. Lake briefly reviews the
inclusions and abnormalities seen in the peripheral blood and bone
marrow of patients with various types of storage diseases. This
discussion is accompanied by good photomicrographs.