Experíence and Reason Briefly Recorded

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Experience

and Reason

. .

Briefly

Recorded

. . .

-“In Medicine one must pay attention not to plausible theorizing but to experience and reason

together.

. . .

I

agree that theorizing is to be approved, provided that it is based on facts, and

systematically makes its deductions from what is observed. . . .

But conclusions

drawn

from

unaided

reason can

hardly

be

serviceable;

only

those

drawn

from

observed

fact.”

Hippocrates:

Preceptz.

. . S

(Short communkationa of factual material are published here. Comments and criticisms appear as Letters to the Editor.)

499 Thrombocytopenic

Purpura

Progress..

ing

to

Transient

Hypoplastic

Anemia

in

a Newborn

with

Rubella

Syndrome

In

the

1963-1964

epidemic

of

rubella

throughout the country a number of infants

presented

with

thrombocytopenic

purpura

usually accompanied

by

leucocytosis

and

a

hemolytic anemia. The bone marrow

exami-nations have shown very few megakaryocytes

but

definite

evidence

of

erythroid

hyper-plasia.14 The patient discussed in this

an-tide presented with hypoplasia of all the

elements of

the

bone

marrow,

including

mega-karyocytes, erythrocytes, and granulocytes.

This represents the second reported instance

of generalized bone marrow depression in the

rubella syndrome and the only surviving

in-fant

with

this

disorder.

CASE HISTORY

R.E. is an 18-month-old Puerto Rican boy born

to a 35-year-old woman who had “German mea-sles” during the second month of gestation. He

was delivered by repeat cesarean section and

weighed 6 ib, 6 oz.

At birth he was considered in excellent

condi-tion, but by 2 hours of age appeared pale with

petechiae and ecchymoses evident over his back and extremities without evidence of gross bleed-ing from the cord or elsewhere. Examination of the eyes did not reveal cataracts, and there was

no evidence of congenital heart disease.

Laboratory values at that time were: hematocrit

48%;

WBC

17,000/mm’;

type

A;

Rh

positive;

Coombs negative. The prothrombin time was

within normal limits. The peripheral blood smear

revealed the presence of normoblasts consistent

with his age, but platelets were completely

ab-sent. Bone marrow examination demonstrated sparse cellularity with normal granulocytic and erythrocytic series, however, no megakaryocytes

were seen.

COURSE

The infant was begun on a course of ACTII

I.M.

followed

by

prednisone by mouth (Fig. 1

shows the outline of the infant’s progress).

At 12 hours of age, the hematocnit dropped to 35% with an increase in petechiae but no gross bleeding. The baby was given a transfusion of

60 cc of fresh whole blood with a rise in the

hematocnit to 52%. Over the first week of life the ecchymoses and petechiae disappeared de-spite little change in the platelet count.

Examina-tion of the bone marrow at 1 week of age

re-vealed rare megakaryocytes. Despite steroids the white-blood cell count and hematocrit continued

to fall. The reticulocyte count failed to rise,

re-maining at 0.8%.

On day 15 a third bone marrow examination

(

Fig. 2.) revealed a sparse marrow containing no

megakaryocytes. Normoblasts were present but

appeared depressed despite the fact that the

hematocrit had fallen to 22%. (The fall in

hema-tocrit should have been enough stimulus to over-come the temporary depression of the bone

mar-row secondary to the transfusion given 15 days

before.) In addition, the granulocytic series showed

toxic granulations and a few vacuoles. The

pie-tune at this time was more consistent with thrombocytopenia, anemia, and neutropenia with

hypoplastic

bone

marrow.

The

infant

continued

to receive steroid therapy, and he required two

fur-ther blood transfusions.

The infant progressively improved and was

discharged at 73 weeks with a hematocrit of 24%;

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RUBELLA

SYNDROME

blood

It’

I

blood

AGE

(weeks)

500

PLATELETS

(1000)

PEMATOCRIT

(%)

w. B.C.

C1000)

POLYS

(1000)

AC TH

mqs.

PREDNISONE

FIG. 1

hemoglobin

8.5

gm/100 ml; RBC 2.86 million/

mm’;

WBC

5,400/mm’

with

a normal

differential;

reticulocyte count 0.6%; and platelet count 32,000/

mm’. The steroid was continued at home. At 23

months of age he was in excellent health. His

hematocrit had risen to 32%, the reticulocyte count was 1.2%, and the platelets had increased to

300,000/mm’. The steroid dosage was tapered over

a 3-week period and discontinued.

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EXPERIENCE

AND

REASON-BRIEFLY

RECORDED

501

readmitted to the hospital at 6 months of age with

bilateral bronchopneumonia. His weight was 6.9 kg (15th l)ercentile); hematocrit 32; WBC 12,250/

mm’; and platelets 300,000/mm. Bone marrow

studies at this time were normal (Fig. :3). An

evaluation of his mental and motor abilities

re-vealed some retardation. His hematological status

had returned to normal. He responded well to treatment and was discharged after 14 days.

The rubella virus was isolated from the Infant’s

throat and urine at 7 months of age. B 9 months

of age the virus could no longer be grown from an source.

Rubella

antibody titers done at that time revealed a titer of 1/64 in the baby’s serum

and a titer of 1/8-1/16 in the mother’s serum.’

DISCUSSION

Prior

to

the

recent

epidemic,

there

were

only two cases of thrombocvtopenic purpura

reported

in infants with rubella

embr’op-athv.

In

these

cases

there

was

no

evidence

of hypoplasia of the granulocytic or

erythro-cytic elements. In the large series reported

within the past year14

the

infants

exhibited

a peripheral leucocytosis and reticulocvtosis

in addition to erythroid hyperplasia in the

bone marrow.

Ervthroid hypoplasia, which was the

un-usual feature in

this

patient,

has

been

recently

reported

in only one other infant with the

rubella syndrome; however, this infant died

at 63 months of age in congestive heart

fail-ure secondary to severe anemia despite a

course of prednisone and testosterone.

In

our

patient

the

hypoplasia

of all the

elements

was

a

temporary

feature

and

re-0 Viral studies were performed b Dr. Louis

Cooper, New

York

Universit’ School of Iedicine,

New York.

cover\’, associated with concurrent therapy, ‘,as complete.

SUMMARY

Amegakaryocytic thrombocytopenic purpura

in

association with

the

unusual

finding

of

a

transient hypoplastic anemia

is reported.

The

hematologic abnormalities completely

disap-peared

despite

the

persistence

of rubella

virus

to age 7

months.

The

influence

of concurrent

steroid administration cannot be evaluated.

CHARLOTTE

Z.

LAFER, M.D.

Department of Pediatrics

ALAN N. MoRRIsoN,

M.D.

Department of Hematology

The

Jewish

Hospital

of

Brooklyn

and

New York State University

Downstate

Medical

Center

Brooklyn,

New

York

REFERENCES

1. Cooper, L., Krugman, S., Giles,

J.,

and Green,

H.

: Personal communication.

2. Rudolph, A.

J.,

Yow, M.D., Phillips, C. A.,

Desmond, M. M., Blattner, R.

J., and

Mel-nick,

J. L.

: Transplacental rubella infection in newly born infants. J.A.M.A., 191:843, 1985.

.3. Horstman, D., et al.: Maternal rubella and the rubella syndrome in infants. Amer.

J.

Dis. Child., 110:408, 1965.

4. Rubella symposium. Amer.

J.

Dis. Child., 110:

1965.

5. Hugh-Jones, K., Mansfield, P. A., and Brewer,

H.

F.

: Congenital thrombocytopenic

pun-pura. Arch. Dis. Child., 35: 146, 1960.

6. Berge, T., Brunnhage, F., and Nilsson, L.:

Congenital h’poplastic thrombocytopenia in

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1966;38;499

Pediatrics

CHARLOTTE Z. LAFER and ALAN N. MORRISON

Experíence and Reason Briefly Recorded

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