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Table

1. Causes

of polycythemla.

Relative polycythemia Dehydration Stress Spurious Tobacco Absolute Pnmary Polycythemia Erythremia Secondary Appropriate High altitude Chronic cardiac polycythemia vera

and

(or) pulmonary

disease

Abnormal chronic

oxygen delivery methemoglobinemia,

to tissues (hemoglobinopathy, 2,3-diphosphogiycerate

deficiency) Obstruction Tobacco Inappropriate Renal cysts Postrenal transplantation of renal blood flow polycythemia

Renal remnants renal disorders (9) Tumors (see Table 2) Other

Essential erythrocytosis; erythrocytosis (10)

familial erythropoietin-dependent

Adapted

from rets.

8-10.

Table

2. Types

of tumor erythropoletln.

reporte d to produce

Malignant tumors Renal adenocarcinoma

Nephroblastoma

(WiIms

Hepatocellular carcinoma Hepatic hemangiosarcoma Testicular carcinoma Malignant pheochromocytoma Mammary carcinoma

tumor)

Nonmalignant

tumors

Hemangioblastoma Cerebellar Brainstem Hepatic Adrenal Meningioma Leiomyoma

obtained trations and its Six plained creasing

free weeks of decreased tendency to support of a-fetoprotein peptide after these were the initial visual to vertigo, possibilities. and of chorionic normal. visit, acuity and headache, the

Serum concen- gonadotropin

patient diplopia; and com- in- an vomiting;

Ophthalmological

examination

suggesting

increased

intracranial

abnormality

was found.

Computer-

re-

and

weight loss.

vealed

papilledema,

pressure.

No retinal

in the of the The ized right fourth tumor primary was revealed nography brain revealed with in slight a metastasis no definite tomography 2). had revealed of erythropoietin, of the hemisphere resulting tomography cerebellar ventricle, a tumor compression hydrocephalus. rather primary and was judged tumor. be than tumor ultraso- to However, a by computerized the abdomen diagnostic serum of the high (Table evaluation concentration Thus, themia, polycy- and or for renal of abdom- a cerebellar hepatic evidence found evidence No nor tumor. dysfunction, was definite any

2094

CLINICAL

CHEMISTRY,

Vol.

40,

No.

11,

1994

inal gery state tumor.

malignancy. in view and the

The

patient

was

referred

for neurosur-

of the

rapid

deterioration

of his clinical

uncertainty

of

the

nature

of

the

cerebellar

During tumor ventral Before performed toms were removed, with par ts surgery, between reported. which reduced neurosurgery, numerous of the a total 6 and Each feeding cerebellar the venous a highly of 10 10 weeks time, vessels was hemisphere phlebotomies after the 400 mL hematocrit vascular

had

been

initial

symp-

of

blood

was

to <48%.

cerebellar located in and the

the cer-

ebellar initial

peduncle. visit, the

Removed tumor contained

in

after

the

from

which

toto 11

weeks

a large

cyst

sampl and course

es

basic was of fluid were fibroblast complicated

taken

for assay

of

erythropoietin

growth

factor.

The

postoperative

by

swelling

of

the cerebellar

hemisphere,

and

probably

also

which

subsequently

after

the operation.

quest,

no autopsy

caused

the

In

accordance

was

done.

by brainstem patient’s with

infarction,

death

1

the

family’s

week re-

Results Pathological-anatomical

examination

of the tumor.

By

light blastoma

microscopy without

distributed

within

the visible

an

tumor

was

mitotic

intricate

activity.

The

cells

were

network

of smaller

and

a capillary

hemangio-

(Fig. 1). of erythropoietin larger capillaries and of basic fibro- al- the phle- in cyst (same that in a and 3, the af- Determinations blast growth factor. Erythropoietin increased concentrations, in relation inappropriately though to during volume, treatment from the nearly (Table cerebellar doubled the erythrocyte botomy fluid 3). The tumor concentration was 7100 IU/L result from in serum previous i.e., 440-fold comparison, the cyst different the same gradient dilutions), time. between several taken case, For at the fluid -190-fold (11). of erythropoietin in Table markedly elimination As shown decreased rate of serum wa concentration s removal of the ter tumor, at a con- admin- did not with to of erythropoietin reasons forming istered recombinant For ethical that humans. we

Fig.

1.

Ught-microscopic

appearance

of

the

cerebellar

tumor.

Large matic larger

tumor

cells are seen, having a foamy

cytoplasm

nuclei, which were distributed

capillaries

(c). van

Gieson

within an intricate stain; bar indicates

and round network of 100 tim.

hyperchro- smaller and

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