Pediatric Annals

EDITORIAL 

Modern Concepts in Pediatric Hematology

Milton I Levine, MD

Abstract

My earliest entrance into the world of pediatric hematology was frightening and almost disastrous. It occurred during my first internship at a children's hospital affiliated with the medical college where we received our training in pediatric surgery. The year was 1927. The hospital was in Hell's Kitchen in New York City. The child was Biagio, a 3-year-old youngster with a greatly enlarged spleen. His skin was yellow and his features were somewhat mongoloid. He was anemic and already had received 15 transfusions. His diagnosis was "Cooley's Anemia" later called Mediterranean Anemia or Thalassemia.

Thomas B. Cooley, a Detroit pediatrician, had described the condition in 1925. He had presented a series of cases of splenomegaly in children with anemia, discoloration of the skin and peculiar changes in the bones.

Biagio was not improving so a splenectomy was performed, and the long vertical incision on the left abdominal wall carefully sutured. The attending surgeon ordered that the stitches not be removed until the 13th day to give assurance of complete healing. On the morning of the 13th day the child's bed was wheeled to the treatment room, and Biagio was lifted up and placed upon the treatment table. In accordance with the procedure at that time, I washed my hands carefully with green soap and rinsed them in alcohol. Then, with my forceps and scissors I proceeded to remove the stitches one by one.

I had removed just Ave of the sutures when suddenly there was a loud, bubbly, gurgling sound, and out of the 3-inch upper portion of the incision the whole mass of the child's small intestines squeezed through and were out on the surface of the body moving around in all directions with the same bubbling and gurgling noise. Instinctively I put my hands down on the intestines trying to force them back through the opening. I realized that my hands were not surgically clean and carried myriad possibilities of infection, but I also realized that if the intestines went cold the little boy would go into shock. I tried my best to force the gurgling mass back, but I was unable to get the twisting coils through the small incision.

I frantically called the nurse but she was paralyzed with fright. I shouted at her to get me warm wet dressings to cover the exposed mass. The nurse turned suddenly, grabbing some gauze pads, held them under the warm sink water, rushed back and threw the wet pads over the exposed intestines. I knew this wasn't the best technique but by then the unsterile procedure was over. I gave Biagio some morphine to quiet him and called by phone for the attending surgeon. He was in the operating room at St. Luke's Hospital miles away. We kept calling surgeons and applying warm wet sterile dressings. One of our attending surgeons finally arrived after 1 and ½ hours and calmly replaced the extruding viscera and once again tightly sutured the wound. Biagio recovered from the ordeal without any difficulty and without any evidence of infection. Remember, this was before the advent of antibiotics.

Today most pediatricians with active practices and especially in large communities have not infrequently met with hematological problems among their patients. Anemia is our most common problem but sooner or later we will meet with cases of thrombocytopenic purpura, leukemia, thalassemia, aplastic anemia, sickle cell anemia and hemophilia. What is our modem approach to such problems?

To answer this we have turned to an outstanding pediatric hematologist who has developed a strong hematological department in the children's hospital of which he is the director.

The Guest…

My earliest entrance into the world of pediatric hematology was frightening and almost disastrous. It occurred during my first internship at a children's hospital affiliated with the medical college where we received our training in pediatric surgery. The year was 1927. The hospital was in Hell's Kitchen in New York City. The child was Biagio, a 3-year-old youngster with a greatly enlarged spleen. His skin was yellow and his features were somewhat mongoloid. He was anemic and already had received 15 transfusions. His diagnosis was "Cooley's Anemia" later called Mediterranean Anemia or Thalassemia.

Thomas B. Cooley, a Detroit pediatrician, had described the condition in 1925. He had presented a series of cases of splenomegaly in children with anemia, discoloration of the skin and peculiar changes in the bones.

Biagio was not improving so a splenectomy was performed, and the long vertical incision on the left abdominal wall carefully sutured. The attending surgeon ordered that the stitches not be removed until the 13th day to give assurance of complete healing. On the morning of the 13th day the child's bed was wheeled to the treatment room, and Biagio was lifted up and placed upon the treatment table. In accordance with the procedure at that time, I washed my hands carefully with green soap and rinsed them in alcohol. Then, with my forceps and scissors I proceeded to remove the stitches one by one.

I had removed just Ave of the sutures when suddenly there was a loud, bubbly, gurgling sound, and out of the 3-inch upper portion of the incision the whole mass of the child's small intestines squeezed through and were out on the surface of the body moving around in all directions with the same bubbling and gurgling noise. Instinctively I put my hands down on the intestines trying to force them back through the opening. I realized that my hands were not surgically clean and carried myriad possibilities of infection, but I also realized that if the intestines went cold the little boy would go into shock. I tried my best to force the gurgling mass back, but I was unable to get the twisting coils through the small incision.

I frantically called the nurse but she was paralyzed with fright. I shouted at her to get me warm wet dressings to cover the exposed mass. The nurse turned suddenly, grabbing some gauze pads, held them under the warm sink water, rushed back and threw the wet pads over the exposed intestines. I knew this wasn't the best technique but by then the unsterile procedure was over. I gave Biagio some morphine to quiet him and called by phone for the attending surgeon. He was in the operating room at St. Luke's Hospital miles away. We kept calling surgeons and applying warm wet sterile dressings. One of our attending surgeons finally arrived after 1 and ½ hours and calmly replaced the extruding viscera and once again tightly sutured the wound. Biagio recovered from the ordeal without any difficulty and without any evidence of infection. Remember, this was before the advent of antibiotics.

Today most pediatricians with active practices and especially in large communities have not infrequently met with hematological problems among their patients. Anemia is our most common problem but sooner or later we will meet with cases of thrombocytopenic purpura, leukemia, thalassemia, aplastic anemia, sickle cell anemia and hemophilia. What is our modem approach to such problems?

To answer this we have turned to an outstanding pediatric hematologist who has developed a strong hematological department in the children's hospital of which he is the director.

The Guest Editor for this symposium is Dr. Philip Lanzkowsky, Chief of Staff and Chief of Pediatric Hematology/Oncology at the Schneider Children's Hospital, Long Island Jewish Medical Center, New Hyde Park, New York. Dr. Lanzkowsky is also Professor of Pediatrics at the State University School of Medicine at Stony Brook, New York. All articles in this symposium on pediatric hematology are from Dr. Lanzkowsky's department.

The first contribution is on the "Modern Management of Thalassemia" and is written by Dr. Robert S. Festa, the Attending Pediatrie Hematologist-Oncologist. What has happened in the past 58 years since Biagio had his splenectomy and proceeded to shock, fluster and throw me into a period of apprehension?

Dr. Festa in his excellent review of the subject brings us the present knowledge with special emphasis on recent treatment. We are all familiar with the bony deformities that characterize this condition and with the cause. Can these deformities be prevented? Then there is the problem of iron overload from the frequent transfusions with the resulting hemochromatosis. Is there any way of effectively removing this excess iron from the body? At what level should a thalassemie child's hemoglobin be maintained, and what program of transfusions is optimal? These are among the numerous subjects considered in this interes'ting and informative article.

The second paper in this review of hematology deals with "Idiopathic Thrombocy topen ic Purpura (ITP) in Children." It is contributed by Dr. Ashok Shende, Attending Pediatrie HematologistOncologist at the Schneider Children's Hospital.

The author first divides the patients into three groups based on degree of thrombocy topen ia - mild, moderate, and severe, the latter having less than 20,000 platelets per mm3. He describes the signs and symptoms in each of these groups. He then considers the marked differences in the acute and chronic forms of the condition. Following a detailed review of the laboratory evaluation of ITP patients, Dr. Shende covers the modern treatment of the disease.

After reviewing the use of corticosteroide and presenting the regimen for their use, he covers the recent exciting use of gamma globulin in children with chronic or intermittent ITP. A study is also reported in which children with the acute condition responded more rapidly to gamma globulin than to corticosteroids. It is further noted that intravenous gamma globulin may be a useful alternative to splenectomy. However, a certain number of children under this treatment fail to gain permanent remission. For these, splenectomy is considered but the risks associated with this procedure are specified. An excellent bibliography covers the subject through 1984.

The next article deals with "Problems in Diagnosis of Iron Deficiency Anemia" and is written by Dr. Philip Lanzkowsky, the Guest Editor of this issue.

This type of anemia is undoubtedly the most common blood abnormality that confronts the practicing pediatrician. The subject is fully covered in this excellent article which takes us logically from the reasons for iron anemia to the action of iron in the body, the methods of accurately diagnosing this deficiency and the optimal treatment. Some interesting facts are brought out in this paper. These include the various stages in the evolution of anemia, the depletion of storage iron, the iron deficient erythropoiesis, and finally, the iron deficiency anemia. Considering these various stages, Dr. Lanzkowsky notes that there are large errors when the Hb level is used as the only test. For improved accuracy, a battery of laboratory measurements are required which are discussed in the article, noting the limitations of each method. The article concludes with the differential diagnosis of iron deficiency anemia.

The fourth contribution to the symposium is on "Current Concepts in the Management of Hemophilia" and is authored by Dr. Gungor Karayalcin, Attending Pédiatrie HematologistOncologist at the Schneider Children's Hospital, and Associate Professor of Pediatrics at the State University of New York at Stony Brook, Stony Brook, New York.

Most practicing pediatricians with fairly large practices sooner or later have cases of hemophilia among their patients. We have experienced the intense suffering of these children from hemarthroses or bleeding in other parts of the body. We also realized the extreme limitations on these youngsters' activities, with no involvement in sports or activities where there might be trauma.

When amniocentesis was established as a means of detecting the male chromosomes, it presented the possibility of terminating the pregnancy if a male fetus of a hemophiliac carrier was detected. This, of course, would mean a sacrifice of many normal infants since there is only a 50% chance that the pathologic condition would be transmitted. However, as Dr. Karayalcin points out in this most interesting and informative article, it is now possible to recognize classic hemophilia in vitro through fetoscopy and fetal sampling and measuring the antigen of factor VIII:C.

In this paper the severity and clinical manifestations of the disease are first presented, and then the various methods of treatment are described with the concentration of factors VIII and IX that they produce. However, hemophiliacs receiving plasma concentrate therapy are at risk for contracting various infections such as hepatitis and AIDS. Dr. Karayalcin reports on the various approaches in combatting these adverse reactions. Among these are the use of heat treated products and the active immunization against Hepatitis B vims.

The article concludes with a detailed study of the optimal treatments for the numerous hemorrhage areas in the bodies of affected children. The author recommends a home treatment program for hemophilic children which has been found feasible and of the greatest benefit in every way. The paper is well organized with excellent tables and bibliography.

The final article is on the important subject of "Marrow Transplantation in Pediatric Hematologic Disorders," and has been written by Dr. Naynesh Kamani of the Division of Immunology and Bone Marrow Transplantation of the Schneider Children's Hospital, New Hyde Park, New York.

Marrow transplantation is a very difficult and time-consuming procedure which, although improved considerably in the past 15 years, is still associated with considerable morbidity and mortality. For not only must the donor-recipient be histo-identical but the transplants, even then, are rejected in over 50% of the cases. The care of the patient prior to transplantation is considerably drawn out and complicated with, as a rule, very high doses of chemotherapy with or without total body irradiation. The harvest material is transfused into the patient intravenously through central venous catheters.

Dr. Kamani notes the high cost of this procedure with its long-term hospitalizaron and the demands on physicians, nurses and the auxiliary supportive staff. But, as a pediatrician who some years ago, was emotionally distraught every time I diagnosed a child with aplastic anemia, I feel deeply that the attempt to cure the condition is worthwhile.

This paper brings us up-to-date on the use and results of marrow transplants in various conditions for which it is presently being used. The author notes that it is the treatment of choice for aplastic anemia, severe combined immunodeficiency and certain cases of acute leukemia.

The reader cannot help but feel that the coming years will bring great advances in this important area of therapy.

10.3928/0090-4481-19850901-04

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