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 book’s 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 treatment recommendations.
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 it’s inside me but isn’t 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-Hodgkin’s lymphoma and Hodgkin’s 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-Hodgkin’s 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 Willebrand’s 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.
A 52-year-old man presented with an erythematous lesion in the axilla of unknown duration. Surgical excision was performed. What is your diagnosis?
