PEDIATRIC
HEMATOLOGY
CARL H. Sr’IITH, M.D., Chairman
IRvING ScHULMAN, M.D., Assistant Chairman
\IARION ERLANDSON, M.D., Secretary
Round
Table
Discussion
678
The purpose of this round table as stated by
Dr. Carl H. Smith was to review those phases
of the anemias of infancy and childhood which
commonly confront the practitioner. It was cm-phasizcd that the approach to a pediatric hema-tologic problem is unique in that a blood dys-crasia must be evaluated against the shifting
i)ioOd levels inherent in the growth of children
ali(I also that it frequently necessitates on
ap-praisal of fetal conditioning factors as well as post-natal hematopoietic influences. Dr. Smith pointed out that many of the essential
diag-nostic procedures, such as a meticulous history and physical examination, the basic blood studies including complete blood count,
retic-ulocyte count and interpretation of the blood
smear, and the comparison of the above studies with the normal range of blood values for each
age group, are well within the working realm
of any pediatric practitioner. An etiological classification of anemias into blood loss, exces-sive blood destruction and hemolysis, bone mar-row depression or infiltration, and deficiency of building materials for hemoglobin or red cell formation was suggested. Reference should be made to the age periods of most frequent
oc-currence. Further specialized procedures were discussed, as well as laboratory tests of which
stool guaiac, serum bilirubin, blood urea ni-trogen, Coomb’s tests, and red cell fragility test were considered to be essential in the in-vestigation of any problematic anemia.
In a discussion of the physiology of neonatal
blood formation and the anemia of
prema-turity by Dr. Schulman, cumulative data from
the literature were shown to illustrate that the
hemoglobin and red cell concentrations in the fetus increase through the second trimester of
intrauterine life and tend to level off during the
third trimester. Since this period is character-ized by a great increase in body size, there must also be an increase in total body
hemo-Presented at the Twenty-Third Annual Meeting of the American Academy of Pediatrics, October, 1954, Chicago, Illinois.
globin. About 82% of the hemoglobin at birth
in premature infants is fetal hemoglobin as
compared to 71% in normal full term infants.
Hemoglobin values immediately after birth are
greatly influenced by the amount of placental blood received at the time of delivery.
During the first 5 weeks of post-natal life
there occurs a steady decrease in total body hemoglobin as a result of loss of both fetal and adult hemoglobins. During this same period there is a steady decrease in the hemoglobin concentration in grams per 100 ml. blood. At
5 weeks of age the body content of adult
hemoglobin begins to increase sharply (reflected
peripherally by a rcticulocytc peak), this gain continuing steadily until the eighteenth week.
Soon after the adult hemoglobin begins to rise,
there also occurs an increase in the body con-tent of fetal hemoglobin. The latter increase, however, is unsustained and shortly thereafter the fetal hemoglobin content again declines steadily. As a result of the gain in adult
hemo-globin the total body hemoglobin content
be-gins to rise and rises steadily to the eighteenth
week.
During this period the hemoglobin
concen-tration levels off despite continuing weight
gain. It requires sixteen weeks for the infant to
regain the hemoglobin mass with which he began life. Since iron derived from the destruc-tion of hemoglobin is not excreted from the body, these sixteen weeks are characterized by
the presence of more iron in the body than is
utilized in hemoglobin synthesis.
Shortly afterward, however, (at 18 weeks) the rate of gain of total body hemoglobin decelerates markedly, accompanied by a fall
in hemoglobin concentration in the blood. With the administration of medicinal iron the rate of gain of body hemoglobin accelerates and the hemoglobin concentration again rises.
In view of these findings the trend in
fac-ROUND TABLE - PEDIATRIC HEMATOLOGY 679
tors as well as hemoglobin concentrations
should be considered in the decision to give
or withhold transfusions. A lowered oxyhemo-globin level as a natural product of the falling hemoglobin concentration was suggested to be a possible stimulus to the resumption of hemo-poietic activity in the anemia of prematurity.
Dr. Smith reviewed the hypoplastic-aplastic
anemias which are characterized by a
normo-chromic and normocytic anemia, absence of
active i)lOOd formation, absence of
enlarge-ment of lymph nodes, liver, and spleen, and
l)y the fact that transfusions are the most
con-sistent therapy. They may be classified as pure red cell anemia, hpoplastic anemias,
(suba-cute as found in non-hemolytic anemia of the newborn, in toxic states, in hemolytic
syn-dromes and in depression following multiple blood transfusions as well as chronic idiopathic or secondary hypoplastic anemia) and aplastic anemia, primary and symptomatic. Many forms of therapy have been tried in the pure red celi
variety including hematinics or vitamins, short wave diathermy and hormones. Transfusions are the only consistent therapy.
A possible etiology of non-hemolytic disease
of the newborn was suggested to be an un-noted hemorrhage into the intervillous spaces
of the placenta. Some such cases have probably
formerly been misdiagnosed as asphyxia pallida
where the hemorrhage was sufficient to cause
a shock-like picture in the infant.
In discussion of several congenital and
hereditary diseases with abnormal hemoglo-bins Dr. Schulman stated that 6 types of
hemoglobin have so far been described; A or
normal adult type, B or sickle hemoglobin,
C, D, E, and F or fetal hemoglobin. It was
pointed out that paper electrophoresis has been
of value in the separation and identification of these hemoglobins. It has been of use in dem-onstrating hereditary aspects of the diseases of which sickle cell anemia is the foremost
example. Fetal hemoglobin is found in
particu-larly high concentrations in Mediterranean
anemia.
It was emphasized that the presence of the
Severe Mediterranean anemia was reviewed
further. As these patients become older there
appears to develop an extracorpuscular factor
resulting in further shortening of the red cell
survival time. This is reflected by an increase
in need for transfusions and often a lowering
of the hemoglobin despite more frequent
trans-fusions. It was shown that in those patients in
The New York Hospital group who reached
this situation and on whom splenectomy was
performed there was noted to be a
post-opera-tive increase in hemoglobin concentration
de-spite the use of many fewer transfusions.
Exact results of splenectomy were
unpredic-table pre-operatively. However in patients
Se-lected according to the criteria above, results
have been encouraging and have been better
in those done near puberty. This is a
treat-ment for one complication of Cooley’s anemia,
namely an acquired cxtracorpuscular factor
de-stroying red cells, and not for the primary disease.
It was also noted that repeated transfusions
in these children as in others may have a
depressant effect on the bone marrow. It might
then be wise to attempt at intervals to wean
the patient from transfusions, especially after
the childhood growth period when the
trans-fusion requirement may be less.
Therapy of the common anemias as outlined
by
Dr. Smith showed iron to be themedica-tion of choice in iron deficiency anemias and
anemia of chronic blood loss. Transfusions are
recommended in ervthroblastosis,
non-hemoly-tic disease of the newborn, anemia due to
hemorrhage and infection and the
hypoplastic-aplastic group with the exception of those
re-suiting from depression following multiple transfusions. ACTH and cortisone may be used in acquired hemolytic anemias and max’
possibly have a place in the therapy of
hypo-plastic anemias and in crises of sickle cell
anemia. Splenectomv is indicated in
sphero-cytic anemias and may be indicated in
Mcdi-terranean anemia, sickle cell anemia and the
hypoplastic-aplastic group in the event that
appar-68() AMERICAN ACADEMY OF PEDIATRICS
arose. Dr. Smith answered that the time of onset is early in the congenital variety and the
Coomb’s test is negative. The acquired form
has later onset with sudden hemolysis and
jaundice, but splenomegaly and sphcrocytes may also be present. There is no significant family history in the acquired form and the
Coomb’s test is positive. The question of
simultaneous crises in several members of one
family having the congenital variety was
cx-plained as possibly due to viremia producing
aplastic crises. Mention was also made that several observers have noted fatalities due to sepsis in splenectomized patients. Although splenectomy is the treatment of choice in
con-genital spheroctic anemia, it was suggested in
view of the above reports that splenectomy be
postponed until after the first several years of
life.
The presence of splenomegaly in iron
de-ficiency anemia was mentioned and Dr. Smith stated it definitely does occur, possibly as a compensatory mechanism to get the greatest possible number of cells into the circulation. It was pointed out that an iron deficiency anemia plus infection can produce a picture of
anemia, splenomegalv and adenitis, which
should not be misdiagnosed as leukemia. The level at which transfusion should be
performed in premature infants was asked.
Out of over 40 cases in the New York
Hos-pital series only one infant fell below 7 grams
when untransfused. Presence of illness and
infection which may interfere with ultimate
bone marrow response, may be indications for
transfusion. The clinical picture appears to be
a better guide than the hemoglobin level.
Therefore, both the patient’s condition and the
hemoglobin concentration must be considered.
In discussion of the use of Roncovite which
contains both cobalt and iron, Drs. Smith
and Schulman felt that the cobalt might induce
a status analogous to anoxemia and therefore
stimulate the baby to make blood before its
natural stimulus occurs, which leaves open the
question of “Is this necessary or desirable?”
The iron contained in the preparation is not
necessary immediately postnatally.
A questioner requested the survival time of
the red cells of premature babies. It is
esti-mated to be about 90 days as compared with
a normal of 120, which shortening would not
be sufficient to explain the anemia of
prema-turity.
In answer to a question relating to type of
blood used in the therapy of hemolytic and
aplastic-hypoplastic anemias, Dr. Smith said
he uses packed red cells in the therapy of all
anemias of this type. Transfusion reactions are
decreased by this procedure. As to frequency
of transfusions, Dr. Smith stated, and Dr.
Roland Scott agreed, that especially in the
early years of rapid growth transfusions should
be liberal in order to keep hemoglobin in a
