TRANSFUSIONS IN PEDIATRIC PRACTICE: INDICATIONS

AND LIMITATIONS
By Carl H. Smith, M.D.
Department of Pediatrics, New York Hospital-Cornell Medical Center

T HE J.NGREASED availability of blood and transfusions yearly. The patients in the lat-
the sharp rise in the number of trans- ten group are treated on an ambulatory
fusions have resulted in the accumulation basis and include largely those with severe
of data by which the indications, limita- Mediterranean anemia and, in smaller num-
tions and risks of this procedure have been bers, sickle cell anemia, pure red-cell
more precisely delineated. The patho- (chronic congenital aregenerative) anemia,
genesis of such complications as serum infants in the convalescent phase of enyth-
hepatitis and hemolytic transfusion reac- roblastosis, hemorrhagic disorders and
tions has been greatly clarified and atten- the anemias associated with leukemia and
tion has been directed to their prevention various systemic diseases. The manifold
and treatment, while other untoward corn- problems that accumulate in this clinic
plications are still under scrutiny. with regard to transfusions parallel in large
An important consequence of these ad- measure those of the inpatient services of
vances has been the growing emphasis on any hospital.
relating a transfusion to a predetermined With the use of available safeguards, the
physiologic need. Except in an emergency, complications from multiple transfusions of
it should ultimately be possible from the blood and less commonly of plasma, such
clinical history, physical examination and as homologous serum jaundice and sensiti-
appropriate laboratory tests to prescribe zation to the lessen blood group factors,
with greater selectivity either whole blood occur less frequently. The major difficul-
or one of its components to supply a de- ties in this clinic relate primarily to choice
ficiency on to correct a pathologic state. of levels of hemoglobin at which blood is to
With expanding knowledge even some of be administered, the almost certain de-
the presumptive “tonic” and “supportive” velopment of hemosiderosis in the chroni-
effects of a transfusion may eventually find cally anemic patient refractory to other
a scientific basis. forms of treatment, and the possibility of
Although the problems relating to trans- depressing intrinsic hematopoiesis by trans-
fusions are common to patients of all ages, fusion.
certain phases of recent developments have
been chosen for special consideration from
SIGNIFICANT FACTORS IN
TRANSFUSION THERAPY
the pediatric standpoint. These are selected
topics in which my associates and I have The approach to transfusion in the pedi-
had a special interest. The observations to atnic patient entails certain preliminaries
be cited are based on study of patients from which scrutinize its need in relation to the
the wards and the Out-Patient Transfusion growth period, potential risks, and tech-
Clinic of the Pediatric Division of The niicaiities of administration. The prepara-
New York Hospital. In this clinic 50 chil- tory considerations, summarized in Table
dren receive a total of approximately 500 I, itlS() provide a basis for the indications

flic data included in this paper are based on studies supported by grants from the National Institute
of Arthritis and Metabolic Diseases, United States Public Health Service (RG A-227 (C5)), The Children’s
11100(1 Foundation, Inc., and Mead Johnson and Company.
Presented in part before the Annual Meeting, October 3, 1955.
ADDRESS: 525 East Sixty-eighth Street, New York 21, Nv York.

596

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 AMERICAN ACADEMY OF PEDIATRICS 597

TABLE I globin and enythrocyte content has, there-

BACKGROUND DATA FOR TRANSFUSION THERAPY fore, come iITltO greater use because it fre-
quently obviates erroneous conclusions
Blood volume-80 mI/kg. drawn from a peripheral blood sample.
Plasma volume-45 mi/kg.
From the blood volume (80 ml./kg. of body
Erythrocyte volume-35 mI/kg.
weight) and hemoglobin measurements (ex-
Hemoglobin concentration-12-13 gin./100 ml.
Hematocrit-36% pressed as gm./100 ml.), the total circulat-
1.0 gm. Hemoglohin=3.4 ng. iron ing hemoglobin mass can be readily deter-
100 ml. Transfused whole blood-average-SO mg. iron mined. In cases in which transfusions are
500 nsl. Transfused whole 1)lood-average-250 115g.
being considered, periodic estimations of
iron
Dosage:
the absolute hemoglobin mass frequently
Whole hlood-20 nil/kg. when body wt. less than prove more valuable in detecting the early
25 kg. regeneration of hemoglobin than determi-
-units of 500 iii!. when I)1)dV wt. iiire
nation of the peripheral hemoglobin con-
than 25 kg.
centration. This calculation, which takes
Packed erythroevtes-15 nsi./kg. wheti body wt. less
than 20 kg. into account the factors of growth and
-units of 80 nil. when body wt. hemodilution, has proved helpful in recog-
more than 20 kg. nizing the initial states of hemoglobin re-
Plasira-10-15 mi/kg.
generation in the premature infant1 and in
evaluating the hemoglobin level prior to
and certain of the limitations of transfusion transfusing the patient with refractory
therapy. chronic hemolytic anemia such as Mediter-
nanean 2 In erythroblastosis, for in-
Blood Volume: Plasma, Total Circulating stance, transfusions can be postponed in
Hemoglobin and Erythrocyte Mass
patients with hemoglobin concentrations of
A knowledge of the shifts in blood volume 8 gm./100 ml. at the fourth to sixth week
has become almost mandatory in the inter- of life in the so-called convalescent anemic
pretation of many clinical syndromes and phases when it is found that the total cm-
in providing clues for their treatment. The culating hemoglobin mass had either sta-
need for recognizing fluctuations of blood bilized or had begun to rise.
volume, and the plasma and erythrocyte With certain exceptions the blood volume
compartments, was early recognized in cited in Table I, applies throughout life.
surgical shock so that adequate physiologic In the newborn the value of 85 ml./kg. has
restitution could be planned. Acute hemor- been found more accurate in calculating the
rhage associated with blood disorders or blood volume prior to administration of
accompanying shock, burns, on organic exchange transfusion. In our experience the
lesions, call when feasible for transfusion blood volume has been found to exceed
of calculated amounts of whole blood, the normal, with values as high as 104
erythrocyte suspensions or plasma. Blood ml./kg. in a small number of patients with
volume measurements now extend into the sickle cell anemia who were not in crisis.
treatment of erythnoblastosis in which an An inordinate increase in plasma volume
exchange of at least twice the estimated was responsible for such values and, on
volume represents current practice. the basis of hemodilution, probably ac-
Comparisons in terms of total henio- counted for the freedom from disability of
globin and erythrocyte mass permit evalu- these patients whose peripheral hemo-
ation of therapeutic procedures or the need globmn values were maintained without
for such procedures by eliminating the in- transfusion at levels of 6 to 7 gm./100 ml.
fluence of variations in plasma volume. The In our experience crisis is associated with
determination of total circulating hemo- a sharp decrease in plasma volume but the

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598 SMITH - TRANSFUSIONS IN PEDIATRIC PRACTICE

amount of hemoglobin undergoes little on with the associated signs and symptoms of
no change. In an attempt to restore the the individual patient. As a guide to man-
precnitical plasma concentration, whole agement we have found that with few ex-
blood, plasma and dextran have been given ceptions, patients with illness in this cate-
with moderate success. gory of chronic anemias do not require
transfusions until hemoglobin levels de-
Hemoglobin and Hematocrit Levels in
crease to 7 to 7.5 gm./100 ml., at which point
Acute and Chronic Anemias
clinical symptoms usually appear. While
In the management of severe anemia, the blood levels in children with this group of
objective in the restoration of a normal diseases tend to decline progressively unless
level of hemoglobin is different in acute treatment is given, the patient with sickle
blood loss than in chronic anemia. In the cell anemia reacts differently. In the latter
patient with nonrecurring hemorrhage, or the need for repeated transfusions is much
in the infant with severe iron deficiency less urgent because hemoglobin levels usu-
who is too ill to await the effects of specific ally stabilize in a range between 6 and 7
therapy, a sufficient amount of whole blood gm./100 ml. without discomfort or inter-
or, preferably, packed erythnocytes, may be ference with activities and regardless of
given to achieve hemoglobin levels of 12 treatment. Although these criteria find gen-
to 13 gm./100 ml. and an hematocnit of 36 eral application, the amounts of blood given
per cent. With severe anemia, preliminary and the intervals between transfusions re-
digitalization may be advisable in the event quine specialized study for each patient.
of elevated venous pressure and other signs
of congestive heart failure. The calculated Transfusions and Hemosiderosis

amount of blood is divided into 2 portions Recent studies in iron metabolism have
to prevent overloading the circulatory sys- extended into an investigation of “iron
tem. Similarly, in erythroblastosis the se- overload” or hemosiderosis resulting from
verely anemic infant is often digitalized, and repeated transfusions. The total amount of
50 ml. of blood withdrawn prior to per- iron in a normal adult approximates 4 to 5
fonmance of exchange transfusion if the gm. of which 15 to 20 pen cent represents
venous pressure is elevated. The transfu- the iron reserve stored principally in the
sion of packed enythnocytes to the patient liven as fennitin and hemosidenin. In chil-
with chronic anemia is to be contrasted dren storage iron normally represents 8.5
with the need for rapid transfusion of whole to 10.5 mg./kg. of body weight. In states
blood in the patient with acute hemorrhage. of iron deficiency in infancy, due to made-
Provided the cause of acute hemorrhage is quate diet and periods of rapid growth,
corrected, the bone marrow will be ade- storage depots are taxed and may be de-
quate to maintain normal blood levels sub- pleted. Iron otherwise accumulates and,
sequently. In chronic hemorrhagic dis- except for the normal excretion of approxi-
orders, completely normal levels of hemo- mately 1 mg. daily, cannot leave the body
globin need not be attained with each except by bleeding. From Table I it is ap-
transfusion if large amounts of blood are parent that excessive amounts of iron from
required to accomplish this end. A normally broken down erythrocytes become avail-
functioning bone marrow will eventually able for storage in patients receiving multi-
raise blood levels to desired values. ple transfusions (.25 gm. iron in 500 ml.
The hemoglobin value at which a trans- blood). Under such conditions hemosiderin
fusion is indicated in patients with anemias deposits in normal storage depots increase
of the aplastic-hypoplastic group or with greatly and, in addition, accessory sites
chronic hemolytic anemias such as severe assume the function of iron storage. An-
Mediterranean aneniia and sickle cell ane- other source of iron stems from the in-
mia, cannot be arbitrarily fixed but varies creased absorption of iron from tile diet

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 ANIERICAN ACADEMY OF PEDIATRICS 599

manifested in patients with chronic refrac- treatment of dehydration, diarrhea and pro-
tony anemia. This phenomenon was strik- tein deficiency. The immunologic support
ingly demonstrated in one of our patients represented by the transfer of circulating
with severe Mediterranean anemia whose antibodies and gamma globulin in combat-
liver at necropsy contained 14 gm. of iron ting infection has now been replaced by the
after receiving only 6.5 gm. by transfusion. antibiotics and, when needed, by the gam-
Whether iron from transfused blood may ma globulin fraction directly.
actually produce hemochromatosis is still The administration of packed enythro-
controversial but there is evidence that ex- cytes, by permitting a larger dose in a
cessive iron storage is potentially injurious.4 single transfusion, represents an ideal treat-
It must be understood that no unequivocal ment in infancy, in patients with cardiac
statement can as yet be made as to the ex- failure, and in chonic anemias refractory
tent on manner in which tissue sidenosis is to other forms of therapy. However, in
harmful. Ourstudies of severe Mediterranean bleeding episodes associated with second-
anemia5 have shown, however, that hemo- any thrombocytopenia due to the bone
siderosis and fibrosis frequently coexist al- marrow depression of aplastic anemia and
though there was no uniformity about the leukemia, especially during chemotherapy,
progression. It has been suggested that the fresh whole blood is preferable presumably
development of true hepatic cirrhosis and for its platelet content. While the trans-
fibrosis of the pancreas which characterizes fen of platelets may not be as effective as
hemochromatosis may depend on the inter- with the use of concentrated platelet sus-
vention of accessory factors such as con- pensions, fresh whole blood is more easily
tinued hypoxia in addition to large iron attainable and frequently controls hemon-
deposits. From these considerations it rhage satisfactorily. Platelet-rich blood from
would seem that the iron derived from an polycythemic donors provides platelets with
occasional transfusion is harmless. A con- a survival span of 4 to 6 days when the
trary situation may prevail, however, in the blood is directly transfused with silicone-
patient receiving multiple transfusions over coated syringes.6 Adnenocorticotropic hor-
long periods of time. In view of the risk mone (ACTH) and cortisone, which can be
involved, the most prudent course is to ne- given intravenously in the emergency,
strict the number of transfusions to the serve as effective hemostatic agents and
minimum compatible with comfort and have supplanted the more cumbersome
moderate activity. methods of providing viable platelets. This
form of therapy is especially useful in idio-
Choice and Dosage of Whole Blood, Packed
pathic thrombocytopenic punpuna in the
Erythrocytes and Plasma
active states and in preparation for splenec-
While the choice and dosage of whole tomy, and also in controlling hemorrhage
blood, packed erythrocytes or plasma are in secondary thrombopenic states.
frequently clear-cut, they often depend on Plasma transfusions restore blood volume
the appraisal of other factors than the when whole blood is not available and con-
levels of hemoglobin and erythrocyte count. rect some clotting deficiencies and supply
A lange body of experience has demon- protein in cases of hypoproteinemia. In
strated the superiority of whole blood, es- true hemophilia, individual units of frozen
pecially when blood volume and anemia group-specific plasma are superior to pooled
require simultaneous correction. Fresh plasma in diminishing the risk of hepatitis.7
whole blood is of value in disorders of the In regulating dosage it should be re-
clotting mechanism by supplying both the membered that citrated blood usually has
appropriate plasma factors and the erythro- a hematocnit of 32 to 36 per cent8 as corn-
cytes lost by hemorrhage. Whole blood, pared with 40 to 50 per cent for whole
plasma, or a combination play a pant in the blood and 60 to 70 pen cent for packed

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600 SMITH - TRANSFUSIONS IN PEDIATRIC PRACTICE

enythnocytes, depending on the amount of begins with the predominant synthesis of

supernatant plasma removed in the latter. the adult type.1 Administration of trans-
In erythroblastosis, for instance, differences fusions at a time when normal negenera-
of hematocnit of recipient and donor, and tion of hemoglobin is at hand seems un-
the effects of swelling of erythrocytes in necessary. Besides the known complica-
acid-citrate-dextrose solution have been ad- tions, to transfuse at this point is to run the
justed by Mollison8 by combining the in- risk of depressing inherent bone marrow
itial replacement with a supplementary ti9 The present trend, as disclosed
transfusion. In that manner the mean hem- in a recent survey50 is to transfuse only
atocnit of Rh positive cells is reduced to those premature infants whose hemoglobin
5 per cent and with the addition of suffi- values have decreased to between 6 and 9
cient Rh negative cells the final total hema- gm./100 ml. Our own practice has been to
tocnit is brought to 50 per cent. transfuse only those whose hemoglobin
The dosages prescribed in Table I serve concentrations persist at levels of approxi-
as guides for the amount of blood and mately 7 to 8 gm./100 ml. without reticulo-
plasma to be given in a single transfusion. cytosis. Transfusion is employed when there
In the case of whole blood and packed are evidences of infection, anorexia, listless-
erythrocytes the values are calculated to ness and failure to gain weight. We have
elevate the hemoglobin concentration and found that premature infants rarely need
enythrocyte count in varying degree depend- transfusions on the basis of anemia alone.
ing on the severity of the anemia but within When required, either packed enythnocytes
safe limits of blood volume. With increas- on whole blood is given in amounts not to
ing weight and expanding blood volume exceed 10 mi/kg. body weight.
these figures lose their significance, and
other criteria are substituted depending on
INDICATIONS FOR TRANSFUSION
the objectives of therapy, such as hemo- The indications for transfusion listed in
globin elevation, cessation of hemorrhage Table II have been dealt with in part in
and clinical improvement. It should be ob- the foregoing analysis of background data.
vious that the olden child with chronic Complete elaboration of each item is be-
anemia who, at irregular periods, receives yond the scope of this paper because such
a single transfusion of 500 ml. of whole elaboration would embrace a detailed con-
blood and lesser amounts of packed eryth- sideration of many hematologic entities and
nocytes cannot be expected to show a ma- of syndromes involving disturbed physi-
tenial rise in hemoglobin. This amount of ology. In pediatric practice the indications
blood is necessarily diluted in a higher apply more frequently to treatment of
blood volume than in the smaller individual
to whom similar amounts may be given. TABLE II
INDICATIONS FOR TRANSFUSION
Hence, in determining the rate of survival
of donor blood, several transfusions are
1. Restore deficit of hemoglobin and erythrocytes:
necessary within a short period so as to Acute and chronic blood loss
raise blood values to normal levels from Hemolytic anemias-congenital and acquired
which the rate of decline may be estimated. Defective blood formation
Pre- and postoperative

Transfusions in Premature Infants . Restore blood volume:

Shock, burns, trauma
In most clinics a conservative policy is 3. Supply specific coagulation factors including platelets
followed with regard to transfusions in the 4. Miscellaneous:

healthy premature infant. At the age of Infections

Hypoproteinemia
4 to 7 weeks, when the infant’s fetal and
Exchange in erythroblastosis
adult hemoglobin values have decreased to Poisoning
low levels, spontaneous recovery usually

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 AMERICAN ACADEMY OF PEDIATRICS 601

blood disorders, infection, nutritional and sists for at least 6 weeks following birth,
deficiency states. In contrast to the situ- a periodic follow-up evaluation appears
ation in the adult, the management of necessary until 3 months of age. Transfu-
transfusion needs in the child requires the sions are unnecessary when hemoglobin
additional appraisal of abnormality in rela- values exceed 8 gm./100 ml. particularly
tion to a specific stage of growth and de- after the first 6 weeks of life, when innate
velopment. Prescribing a hemoglobin level, synthesis is in progress.
for instance, to be achieved by transfusion
TRANSFUSION THERAPY IN
may not be a simple task in the infant or
HEMORRHAGIC DISORDERS
young child because the optimal value is
not fixed but is subject to individual inter- The intensive investigations of the hemor-
pretation. rhagic disorders have led to a clearer defi-
The complexities of establishing a uniform nition of the role of established and newly
transfusion routine are exemplified in the discovered factors in maintaining hemo-
modern treatment of erythroblastosis. Al- stasis. This expansion of knowledge has
though many related sources of informa- created a complexity in concepts dealing
tion aid in arriving at a decision for ex- with the steps of coagulation and their in-
change transfusion one of the most im- tegration. With the application of spe-
portant is the hemoglobin concentration of cialized procedures, defects heretofore,
cord blood. Yet, in various clinics, values unknown have been described and appro-
from as low as 12 to as high as 15 gm./100 pniate treatment advised. The 2 clotting
ml. are considered indication for treatment. factors required for thromboplastin genera-
A similar diversity of opinion, though less tion, plasma thromboplastin component
important, concerns the hemoglobin value (PTC) and plasma thromboplastin ante-
to be maintained in the various stages of cedent (PTA), and factors V (labile fac-
the disease: during the hospital stay fol- ton) and VII (stable factor) for prothnombin
lowing exchange transfusion, prior to dis- conversion are recent developments. Ten
charge, and until the infant is approxi- to twenty pen cent of cases of hemophilia
mately S months of age. With the technical are now ascribed to a deficiency of FTC
facilities now available, it seems justifiable rather than of antihemophilic globulin.11
to use the higher values as an indication The bleeding condition classified as von
for performing exchange transfusions when Willebrand’s syndrome on pseudohemo-
supported by other related factors. With philia has been subdivided into 2 types:
an exchange transfusion and 1 supplemen- one restricted to a vascular defect, and, in
tary transfusion some infants pursue a our experience,12 another more common
benign course with hemoglobin levels of 10 type which occurs in combination with
to 12 gm./100 ml. and enythnocytic counts varying degrees of deficiency of antihemo-
of 3,000,000 to 3,500,000/mm.3. In others, philic globulin.
in whom the hemolytic process is more in- As the onset of the majority of these dis-
tense, a second exchange transfusion and/or orders occurs in infancy and childhood,
several supportive transfusions are neces- the pediatrician is usually expected to di-
sary to reach these levels. In any event, any rect the initial studies, and to supervise
decline below this range prior to discharge treatment after the nature of the defect
is an indication for a single transfusion of has been thoroughly investigated. For most
packed erythrocytes. Raising the hemo- bleeding conditions the history, physical
globin to approximately 12 to 14 grn./100 examination and a few simple tests will
ml. at this time offsets the development of reveal whether the pathogenesis is based
the extremely low levels which occasionally on a reduction of platelets, abnormality of
occur in the first weeks after discharge. vascular function or of the clotting mecha-
With the knowledge that Rh antibody pen- nism. Although these hemorrhagic dis-

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602 SMITH - TRANSFUSIONS IN PEDiATRIC PRACTICE

TABLE III TABLE IV

TRANSFUSION THERAPY IN THE HEMORRhAGIC LIMITATIONS AND HAZARDS OF BLOOD TRANSFUSIONS

DISORDERS
1. Development of intra-group isoimmunization

A. Fresh whole blood or plasma: . Ilemolytic reactions:

Thrombocytopenic purpura A. Incompatible blood
Ilelnophilia (hemophilia A, AuG deficiency) B. Old stored blood
Parahemophilia (factor V deficieiwy) 3. Trahislnission of disease:
Von W’illebrand’s disease A. homologous serum jaundice

I . Vascular defect only (pseudoheinophilia) B. Syphilis

2. Vascular defect with AHG deficiency (vascular C. Malaria
hemophilia) 4. Circulatory overload
Afibrinogenemia 5. Excessive iron deposition:
B. Fresh or stored whole blood or plasma: A. Hemosiderosis
Prothrombin deficiency B. Exogenous hemuochromatosis
IyrC deficiency (hemophilia B, Christmas factor) 6. Suppression of hematopoiesis
PTA deficiency 7. Miscellahwous:
Factor VII deficiency (stable factor) Febrile reactions
Allergic reactions
Reactions from infected blood
orders are classified in Table III according Potassium toxicity
to the requirements for fresh or stored Cold agglutinins
whole blood on plasma, patients in either Air embolism
Citric acid intoxication
category would benefit from fresh whole
blood. It is important to emphasize that in of techniques and identification of incneas-
such hemorrhagic disorders as thrombo- ing numbers of rare blood factors.
cytopenic purpura, hemophilia, vascular One of the limitations of transfusion
hemophilia and afibninogenemia, too fre- which deserves comment and which has as
quent transfusions of whole blood or plasma yet received scant attention is its potential
may induce the formation of antibodies to depressant effects upon endogenous eryth-
platelets, to antihemophilic globulin or to ropoiesis and hemoglobin synthesis. That
fibninogen, thus counteracting the benefi- transfusions possess this secondary inhibi-
cial effects of treatment. tory effect was recognized in the treatment
of pernicious anemia before the advent of
LIMITATIONS AND HAZARDS
specific therapy and has been confirmed
OF TRANSFUSIONS
more recently in several studies.1417
Increased experience and investigation While retardation of hematopoiesis may
of untoward reactions have provided a in minor degree accompany a single trans-
greater measure of safety in the administra- fusion, it is overshadowed by the major
tion of transfusions. Of the limitations and corrective effects. In the case of multiple
hazards listed in Table IV, the most corn- transfusions, however, this limiting influ-
mon are the hemolytic reactions, serum ence may explain the failure of transfusion
hepatitis, allergic and febrile reactions, and to produce an anticipated rise in hemo-
circulatory overload. Many advances have globin and enythrocytes in the absence of
been made in the direction of reducing the hemolysis or hemorrhage. Two sets of ob-
icterogenic properties of plasma such as servations from our laboratory18 document
reduction of the size of the pool, ultra- the thesis that transfusions administered at
violet irradiation, and the storage of liquid frequent intervals retard hemoglobin and
pooled plasma for prolonged periods at erythrocyte formation. These data are
room temperature.11 The incidence of iso- based on an intensive study of a small
sensitization from blood transfusion has group of children with severe Mediter-
been markedly reduced by preliminary ranean anemia in whom transfusions could
screening and cross-matching, refinements be interrupted following splenectomy. By

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 AMERICAN ACADEMY OF PEDIATRICS 603

methods designed to permit quantitative SUMMARY

differentiation between donor and recipient
blood and by serial examinations of the total The effectiveness of transfusion of whole
erythrocyte mass and circulating hemo- blood or any of its components depends
globin it was possible to demonstrate that in large measure on a clean understand-
a retarding effect occurred which was most ing of the anticipated role in supply-
marked from the first to third week fol- ing a specific deficiency or correcting a
lowing transfusion. Not until donor blood pathologic state while realizing the limita-
had been entirely eliminated from the tions and hazards.
circulation were pretransfusion blood levels A prerequisite in infants and children is
restored in the patient. a knowledge of the normal changes occur-
The depressant effect of transfusion was ing at various developmental periods so as

further substantiated by another means. In to appraise the abnormality more accurately

severe Mediterranean anemia the presence and to determine the need and extent of
of large amounts of fetal hemoglobin pro- reparation.
vides a biologic tag of endogenous hemo- The pathogenesis of various bleeding dis-
globin synthesis. Transfusions resulted in orders has been more precisely character-

a depression of this component which in- ized so that treatment can be prescribed
creased sharply as donor cells left the circu- with greater selectivity.
lation. The diminution in circulating fetal Except during active hemorrhage, hold-
hemoglobin persisted in 1 patient for the ing blood requirements to a minimum fne-
first 7 weeks after transfusion and in 2 quently serves the patient to greater ad-
others the decrease amounted to 50 pen vantage than maintaining normal hemo-
cent. Paralleling these observations is a re- globin levels by frequent and maximal ad-
cent report1#{176} that in patients with sickle ministrations of blood. This restriction is
cell anemia receiving multiple transfusions, especially pertinent in the refractory ane-
a period of maximum depression of eryth- mias-the aplastic-hypoplastic and chronic
ropoiesis occurred from the twelfth to the hemolytic groups. Here multiple transfu-
twenty-fifth day, the percentage of sickle sions result not alone in hemosiderosis but
cells decreasing from 100 to 5 pen cent. in a potential suppressive effect on erythro-
The conclusions drawn from these studies poiesis and hemoglobin synthesis. The re-
do not detract from the necessity of trans- tanding effect of transfusion must also be
fusing patients with chronic anemia, but considered in the management of the con-
emphasize the advisability of occasionally valescent anemic phase of erythroblastosis
withholding treatment to determine in- and in the anemia of prematurity.
nate bone marrow function. In critical It should be emphasized that the hemo-
periods of growth such as in the anemia of globin concentration at which transfusion
prematurity, it may be undesirable to inter- is indicated for patients who require single
fene with bone marrow function by trans- on multiple administrations of blood cannot
fusion. In enythnoblastosis it has been sug- be arbitrarily fixed but varies with the at-
gested2#{176} that a similar retardation can result tendant clinical signs and symptoms and the
from a persistent effort to maintain normal promise of relief by this form of treatment.
blood levels. These studies suggest the
need for a less empirical and more in- REFERENCES
dividualized orientation, not alone in ne-
fractory anemias but in other disease con- 1. Schulman, I., Smith, C. H., and Stern,
C. S. : Studies on the anemia of prema-
ditions requiring frequent transfusions. The
turity. Am. J. Dis. Child., 88:567, 1954.
spacing and size of transfusions and blood
2. Smith, C. H., Schulman, I., Ando, R. E.,
levels to be attained require constant ne- and Stern, C. : Studies in Mediterranean
examination and appraisal. (Cooley’s) anemia. 1. Clinical and hema-

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604 SMITH - TRANSFUSIONS IN PEDIATRIC PRACTICE

tologic aspects of splenectomy, with spe- familial hemorrhagic disease in males

cia! reference to fetal hemoglobin syn- and females characterized by combined
thesis. Blood, 10:582, 1955. anti-hemophilic globulin deficiency and
3. Pickles, M. M. : Haemolytic Disease of the vascular abnormality. Am. J. Dis. Child.,
Newborn. Springfield, Thomas, 1949, in press.
p. 106. 13. Allen, J. C., Emerson, D. M., Barron,
4. Dubin, I. N. : Idiopathic hemochromatosis E. S. G., and Sykes, C. : Pooled plasma
and transfusion siderosis; a review. Am. with little or no risk or homologous
1. Clin. Path., 25:514, 1955. serum jaundice. J.A.M.A., 154:103,
5. Ellis, J. T., Schulman, I., and Smith, C. H.: 1954.
Generalized siderosis with fibrosis of 14. Robertson, 0. H. : The effects of experi-
liver and pancreas in Cooley’s (Medi- mental plethora on blood production.
terranean) anemia, with observations on J. Exper. Med., 26:221, 1917.
the pathogenesis of the siderosis and 15. Dacie, J. V. : Transfusion of saline-washed
fibrosis. Am. J. Path., 30:287, 1954. red cells in nocturnal haemoglobinuria
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 TRANSFUSIONS IN PEDIATRIC PRACTICE: INDICATIONS AND
LIMITATIONS
Carl H. Smith
Pediatrics 1956;17;596

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Pediatrics is the official journal of the American Academy of Pediatrics. A monthly publication, it has
been published continuously since . Pediatrics is owned, published, and trademarked by the American
Academy of Pediatrics, 141 Northwest Point Boulevard, Elk Grove Village, Illinois, 60007. Copyright ©
1956 by the American Academy of Pediatrics. All rights reserved. Print ISSN: .

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 TRANSFUSIONS IN PEDIATRIC PRACTICE: INDICATIONS AND
LIMITATIONS
Carl H. Smith
Pediatrics 1956;17;596

The online version of this article, along with updated information and services, is located on
the World Wide Web at:
http://pediatrics.aappublications.org/content/17/4/596

Pediatrics is the official journal of the American Academy of Pediatrics. A monthly publication, it has
been published continuously since . Pediatrics is owned, published, and trademarked by the American
Academy of Pediatrics, 141 Northwest Point Boulevard, Elk Grove Village, Illinois, 60007. Copyright ©
1956 by the American Academy of Pediatrics. All rights reserved. Print ISSN: .

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