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Medical Forum / Diseases and Disorders / Lupus / November 2007

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Scleroderma and  Erythrocytosis

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ironjustice@aol.com - 14 Nov 2007 03:15 GMT
Scleroderma is a connective tissue disease thought to be related to
increased red blood cell production / erythrocytosis /
hemochromatosis / polycythemia.

Mt Sinai J Med. 2006 May;73(3):620-1.Links
Erythrocytosis in a scleroderma patient.
Mabo E, Edeki T, Westney GE, Reed JW, Guo X.
Department of Internal Medicine, Morehouse School of Medicine,
Atlanta, GA 30310, USA.

A 40-year-old black male with scleroderma lung disease presented with
blurry vision and headache. His presenting hemoglobin was 22.3 g/dL
and his serum erythropoietin level was surprisingly low. Although
nocturnal hypoxemia was evident, his daytime resting arterial oxygen
saturation was normal. The patient's symptoms of hyperviscosity
improved after phlebotomy, as his hemoglobin gradually decresed to
18.3 g/dL. Repeat serum erythropoietin levels were in normal and high
ranges. Patients with chronic interstitial lung disease and
erythrocytosis could have normoxemia at rest and a normal or low serum
erythropoietin level at the peak of erythrocytosis. A repeat sampling
of serum erythropoietin and monitoring of oxygen saturation during
sleep and exertion may help in diagnosis. Physicians should prescribe
continuous oxygen therapy for patients with chronic interstitial lung
disease and erythrocytosis, even if diurnal resting hypoxemia is
absent.

PMID: 16758101 [PubMed - indexed for MEDLINE]

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ironjustice@aol.com - 14 Nov 2007 04:04 GMT
On Nov 13, 7:15 pm, "ironjust...@aol.com" <ironjust...@aol.com> wrote:
Scleroderma is a connective tissue disease thought to be related to
increased red blood cell production / erythrocytosis /
hemochromatosis / polycythemia.<<

Graft versus host disease is **indistinguishable** from iron
overload .. and coincidentally .. it presents .. scleroderma.

Hemosiderin deposits in chronic graft-vs.-host disease related
myopathy
Authors: Schmidt-Hieber, Martin1; Okuducu, Ali Fuat2; Stoltenburg,
Gisela2; Mackert, Bruno-Marcel3; Benzian, Nadia4; Thiel, Eckhard1;
Blau, Igor Wolfgang1

Source: European Journal of Haematology, Volume 75, Number 6, December
2005 , pp. 522-526(5)

Publisher: Blackwell Publishing

Abstract:

Schmidt-Hieber M, Okuducu AF, Stoltenburg G, Mackert B-M, Benzian N,
Thiel E, Blau IW. Hemosiderin deposits in chronic graft-vs.-host
disease related myopathy.

Eur J Haematol 2005: 75: 522-526. ? Blackwell Munksgaard 2005.
Abstract:

Chronic graft-vs.-host disease (cGVHD) occurs in 20-50% of patients
who survive for at least 100 d after allogeneic stem cell
transplantation (SCT). cGVHD includes scleroderma-like skin changes,
chronic cholangitis, obstructive lung disease and general wasting
syndrome. Polymyositis or myopathy are rare manifestations of cGVHD
with approximately 40 reported cases. Polymyositis accompanied by
hemosiderin deposits in cGVHD has been reported only once, and there
are no reports on lipofuscin deposits in skeletal muscle cells in
cGVHD. We report here on a 56-yr-old male who underwent allogeneic SCT
in 1999 for osteomyelofibrosis and progressive hematopoietic
insufficiency. In February 2004, the patient was hospitalized for
progressive muscular weakness with loss of the ability to walk.
Laboratory tests demonstrated normal values for serum creatine kinase,
aldolase and lactic dehydrogenase; the ferritin level was highly
elevated. The femoral muscle biopsy showed mostly perifascicular
atrophy as well as numerous subsarcolemmal hemosiderin and lipofuscin
deposits. Intravenous administration of the chelating agent
deferoxamine was ineffective. Three weeks later the patient died of
aspiration pneumonia. Interestingly, autopsy disclosed moderate
hemosiderin deposits in the liver, the organ usually involved in
hemosiderosis.
Keywords: stem cell transplantation; GVHD; myopathy; hemosiderin;
lipofuscin

Document Type: Research article

DOI: 10.1111/j.1600-0609.2005.00548.x

Affiliations: 1: Medizinische Klinik III (H?matologie, Onkologie und
Transfusionsmedizin), Charit?- Campus, Benjamin Franklin,
Hindenburgdamm, Berlin, Germany 2: Institut f?r Neuropathologie,
Charit?- Campus Virchow-Klinikum, Augustenburgerplatz, Berlin 3:
Klinik f?r Neurologie, Charit?- Campus Benjamin Franklin,
Hindenburgdamm, Berlin 4: Institut f?r Pathologie, Charit?- Campus
Benjamin Franklin, Hindenburgdamm, Berlin

Who loves ya.
Tom

Jesus Was A Vegetarian!
http://jesuswasavegetarian.7h.com

Man Is A Herbivore!
http://tinyurl.com/a3cc3

DEAD PEOPLE WALKING
http://tinyurl.com/zk9fk

> Mt Sinai J Med. 2006 May;73(3):620-1.Links
> Erythrocytosis in a scleroderma patient.
[quoted text clipped - 28 lines]
>
> DEAD PEOPLE WALKINGhttp://tinyurl.com/zk9fk
 
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