Hereditary cryohydrocytosis with normal stomatin

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ORPHA:398088OMIM:185020D58.8
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1Active trials8Treatment centers

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Overview

Hereditary cryohydrocytosis with normal stomatin is an extremely rare inherited blood disorder that affects red blood cells. In this condition, red blood cells become abnormally leaky, especially when exposed to cold temperatures. The cells take in too much water and swell up, which can cause them to break apart — a process called hemolysis. This leads to a type of anemia known as hemolytic anemia. The word 'cryohydrocytosis' comes from 'cryo' (cold), 'hydro' (water), and 'cytosis' (relating to cells), reflecting how cold temperatures worsen the water leakage into red blood cells. The term 'with normal stomatin' distinguishes this form from a related condition where a protein called stomatin is missing from the red blood cell membrane. Patients may experience symptoms of anemia such as fatigue, pale skin, and jaundice (yellowing of the skin and eyes). Episodes of red blood cell breakdown may be triggered or worsened by cold exposure. Some patients may also develop an enlarged spleen because the spleen works harder to filter damaged red blood cells. The severity can vary between affected individuals. Treatment is mainly supportive, focusing on managing anemia and avoiding cold exposure when possible. In some cases, blood transfusions may be needed during severe episodes. Splenectomy (surgical removal of the spleen) has been considered in some patients but must be approached with caution, as it may increase the risk of blood clots in this condition.

Key symptoms:

Fatigue and tirednessPale skinJaundice (yellowing of skin and eyes)Dark-colored urineEnlarged spleenEpisodes of anemia worsened by coldShortness of breath during exertionGallstonesRapid heartbeatWeaknessCold sensitivity

Inheritance

Autosomal dominant

Passed on from just one parent; each child has about a 50% chance of inheriting it

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗OMIM ↗NORD ↗

FDA & Trial Timeline

1 event
May 2021National Exhaustive Cohort of Hereditary Stomatocytoses and Other Channelopathies Affecting the Red Blood Cell

Assistance Publique - Hôpitaux de Paris — NA

TrialRECRUITING

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Treatments

No FDA-approved treatments are currently listed for Hereditary cryohydrocytosis with normal stomatin.

1 clinical trialare actively recruiting — trials can provide access to cutting-edge therapies.

View clinical trials →

Clinical Trials

1 recruitingView all trials with filters →
N/A1 trial
National Exhaustive Cohort of Hereditary Stomatocytoses and Other Channelopathies Affecting the Red Blood Cell
N/A
Actively Recruiting
PI: Corinne GUITTON (APHP) · Sites: Le Kremlin-Bicêtre

No specialists are currently listed for Hereditary cryohydrocytosis with normal stomatin.

View NORD Rare Disease Centers ↗Undiagnosed Disease Network ↗

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Hereditary cryohydrocytosis with normal stomatin.

Search all travel grants →NORD Financial Assistance ↗

Community

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Caregiver Resources

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Questions for your doctor

Bring these to your next appointment

  • Q1.How severe is my (or my child's) anemia, and how often will blood tests be needed?,What specific precautions should I take regarding cold exposure?,Should I take folic acid or any other supplements?,Is there a risk of iron overload, and how will it be monitored?,Why is splenectomy not recommended, and what are the risks?,Are there any activities or situations I should avoid?,Should other family members be tested for this condition?

Common questions about Hereditary cryohydrocytosis with normal stomatin

What is Hereditary cryohydrocytosis with normal stomatin?

Hereditary cryohydrocytosis with normal stomatin is an extremely rare inherited blood disorder that affects red blood cells. In this condition, red blood cells become abnormally leaky, especially when exposed to cold temperatures. The cells take in too much water and swell up, which can cause them to break apart — a process called hemolysis. This leads to a type of anemia known as hemolytic anemia. The word 'cryohydrocytosis' comes from 'cryo' (cold), 'hydro' (water), and 'cytosis' (relating to cells), reflecting how cold temperatures worsen the water leakage into red blood cells. The term 'wi

How is Hereditary cryohydrocytosis with normal stomatin inherited?

Hereditary cryohydrocytosis with normal stomatin follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

Are there clinical trials for Hereditary cryohydrocytosis with normal stomatin?

Yes — 1 recruiting clinical trial is currently listed for Hereditary cryohydrocytosis with normal stomatin on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.