First announcement of the Fanconi anemia ...

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1982 60: 1054

First announcement of the Fanconi anemia International Registry [letter] AD Auerbach and TM Schroeder

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From bloodjournal.hematologylibrary.org by guest on October 16, 2011. For personal use only.

CORRESPONDENCE

First To the

Announcement

Fanconi

Arleen

D.

West

Germany,

order

to establish

known

and

our

method

based

amniotic

fluid

(Auerbach gather

genetic,

define

the

hematologic,

and

heterogeneity

differential

diagnosis,

including

the incidence

and

study

study.

aware

of patients

aim

data

of donor

Clinic

reports.

blood

without

trouble

with

that

and

efficient

Doing

fewer

money

but

disease,

clinicians

and

hematology

physicians

to join

and scientists

test

other

at

tests

as pointed

most

out

Assistant The

York

New

Professor

E. Rosenfield, ofPathology

In

York

1982),

S.P.

review

To the

Masouredis

Med.

number Genetics

paper

of Heidelberg

Neuenheimer Heidelberg Germany

Dr.

Rosenfield’s

of transfused

recipients

cited

mediated

Feld

West

328

by Rosenfield, cell

Washington,

et al. (American remarking at risk and

for delayed

basis

of

Taswell

units

of

hemolysis.”

patient

transfused

blood

however, tion,”

Clinic

Taswell

(“A

America ,

cations fusion

pp 71-92)

seminar

with

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the

incidence

increased

from

Blood

indicates

over

I in 400

transfused

more figures

and

me that

4 units Banks,

units

transfused Second,

cell

destruc-

Washington,

of identified

Medicine CUNY

Department and

of

Clinical

Sinai

Microscopy

Hospital

York, N. Y.

administered

reactions

manifested

(1964-1973) The

patients

at Mayo

and,

in fact,

have

estimate

delayed seminar of

and did

not quote

by serologic

changes.

To

attention

only

come

positive

to our

antibody

no evidence

Blood

an incidence

incidence,

screen

and

of hemolysis

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on immune-

reported This

have

of the

hemolysis.

Association

of I : I 500 units only

of my

for

et al. (“A

pp 71-92)

per patient.

patients

developed

correction at risk

Banks, of post-

the number

however,

includes

quote,

direct

“Some

because

of a

Coombs

test

or shortened

blood

cell

hemolysis

in

survival.”

The

difficulty

transfused namely,

that

required

for

difficulty

with

my

concluding

compatibility and

effective

defining

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risks

of

illustrates

the

thesis

developed

in

patients

assessing

the

compatibility Dr.

remarks

testing in

final

point, out for

my

that

I have

I reference

the current if we had

predicting

in

review,

procedures

problem.

since

be unnecessary

methods

in

laboratory

is a complex

by pointing would

vitro

appropriate

testing

Rosenfield’s

clinical

no

him

in

debate more

vivo

on

reliable red

cell

survival.

D.C., communi-

hemolytic

(1978-1980).

likely Howard

blood.

improved

be

on the

Dr.

each

of

continuingly

1:6200

to 1:1400

incorrect

the

immune-mediated

of

then

1054

on

that,

and

that

of

Association

(1974-1977)

Bank

American

1981,

hemolysis

of units

by

was both

et al. gave told

average

showed

at Mayo reactions

an

1980) may

not patients,

has personally

Moore

recipients

understated

and

ofS.B.

74:94-97,

(0.025%)

Moore

59:873-875,

report

interpretation

gravely First,

receives

Clinic

(Blood

Pathology

This it

problem.

of Mayo

Mayo

Mayo

up to I in 4000

because

of the

tests

ofClinical

“possibly

misleading

magnitude

1981

Journal

that

the

Emeritus,

Taswell,

destruction,” D.C.,

transfusion

pretransfusion

cited

and ofMedicine,

Editor:

I appreciate

ofHuman

6900

of

of hemolytic

10021

recently

brief

and

the rug until

M.D.

School

Mount

New

Editor his

Sinai

Blood

asymptomatic To the

save time

recognition

Richard

The

University

Dr.

University Im

will under

Professor Director

Ph.D.

M. Schroeder

Institute

Much needed.

Avenue

York, New

Traute

think.

are badly

us

who are

Investigative Dermatology

Rockefeller

/230

cross-

institutions,

bankers

by Masouredis, their

not have It is clear and

other

problem

Mayo

reactions.

Professor

Laboratoryfor

would

but they

how to improve

by

screening

blood

of the

Rh-type-specific

patients.

most

desirable,

Mount D. Auerbach,

made and

both

and

this critical

is invited. Arleen

in

by

to determine

we probably

Clinic

less than

learn

ABO

technical given

indictment

specifically

tests

also sweep

finally

a serious

issued

testing,

Mayo

or value

1 in 200 transfused

are not only

tests, it will

clerical

wrong

procedures

a point

than

considerably

of exact

popular

antiglobulin

as done

more

of the

any

the

represents

if we just

more

to

from

also

blood,

all,

doing

albumin

accomplish

course

After much

transfusion

from

FA

compatibility

Registry

unrelated

fold

figure

of currently

is

to eventually

the

ten

the sensitivity

to

a means

by

But

patients,

and

reaction

differs

matching,

breakage

in order

the clinical

and

This

of the registry

FA

establish

in genetics

Correspondence with

is

and

of malignancy.

We ask all of our colleagues in this

FA

fibroblasts,

chromosome

presumptive clinical

of

all

of the features

lymphocytes,

in the disorder,

of

use of a cytogenetic

67: 128, 198 1 ). The all

Masouredis.

of patients

some

by the induced

as possible,

with

diagnosis

diversity

manifest

of FA

error.

us in

International

transfusion

Schroeder,

Registry

Clinical

that

to chemically

as far

International

be confirmed

on the sensitivity

M.

to cooperate

patients.

disorders

may cells

Traute

due to phenotypic

et al. Pediatrics

to ascertain,

an

(FA)

of other

Diagnosis

and

colleagues

maintain

to establish

the occurrence

U.S.A.,

upon

anemia

difficult

FA.

Auerbach,

call

Fanconi

often

Anemia hemolytic

Editor.

We,

of

of the

transto 1:3400

latest Clinic

figure has a

S. P. Masouredis,

M.D.,

Professor

of Pathology

Director,

University

University La Jolla,

Hospital

ofCalifornia. Calif

Blood,

Ph.D.

San

Blood

Bank

Diego.

T-003

92093

Vol. 60, No. 4 (October).

1982