Disease Directory Pearson Syndrome
Mitochondrial

Pearson Syndrome

Also known as: Pearson marrow-pancreas syndrome, sideroblastic anemia with exocrine pancreatic dysfunction

Prevalence

Fewer than 100 cases reported; extremely rare

Onset

Neonatal / Infancy

Type

Rare mitochondrial disease

Gene

mtDNA deletion

About Pearson Syndrome

Pearson syndrome is a rare, usually fatal disorder of infancy caused by large-scale deletions of mitochondrial DNA, characterised by refractory sideroblastic anaemia with vacuolisation of marrow precursors and exocrine pancreatic dysfunction. Children who survive the haematological phase often show partial haematopoietic improvement, but may subsequently develop features of Kearns-Sayre syndrome as the mitochondrial deletion persists in other tissues. Supportive care, including transfusions and pancreatic enzyme replacement, is the mainstay of management.

Common Clinical Features

Transfusion-dependent sideroblastic anaemia in infancy Vacuolisation of bone marrow precursors Exocrine pancreatic insufficiency with malabsorption Lactic acidosis Hepatic dysfunction Failure to thrive Risk of progression to Kearns-Sayre syndrome

Clinical Trial Eligibility Tips

What to know before applying to Pearson Syndrome trials.

Bone marrow biopsy confirming vacuolisation and ringed sideroblasts, together with mtDNA deletion analysis, are required for definitive diagnosis and trial enrolment.

Due to extreme rarity, trials may be conducted under compassionate use or expanded access frameworks; contact the treating centre and the UMDF for guidance on access programmes.

Transfusion dependency and haematological status at the time of screening are key eligibility variables; maintain accurate transfusion records.

Patient Resources

Patient Organization

United Mitochondrial Disease Foundation

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Orphanet

European reference resource for rare diseases (ORPHA:699)

View on Orphanet ↗

NORD

National Organization for Rare Disorders

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Find recruiting Pearson Syndrome trials

Search 500,000+ studies from ClinicalTrials.gov, filtered for Pearson Syndrome. Updated daily.

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