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dehydrated hereditary stomatocytosis - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

dehydrated hereditary stomatocytosis

MONDO:0017910

Also known as:hereditary xerocytosisDesiccytosis hereditaryxerocytosis hereditary

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Overview

Dehydrated hereditary stomatocytosis (DHS) is a rare hemolytic anemia characterized by a decreased red cell osmotic fragility due to a defect in cation permeability, resulting in red cell dehydration and mild to moderate compensated hemolysis. Pseudohyperkalemia (loss of potassium ions from red cells on storage at room temperature) is sometimes observed.

OnsetCONGENITAL

Clinical Trials1 registered · 1 recruiting

Clinical Presentation

Built from Human Phenotype Ontology (HPO) phenotype annotations and GeneReviews clinical descriptions. Features represent findings documented across known cases — not an exhaustive list of possible symptoms.

Features include very common findings: Red blood cell destruction (hemolytic anemia), Nonspherocytic hemolytic anemia, and Increased red cell osmotic fragility; and common findings: Cholelithiasis, Enlarged spleen (splenomegaly), Increased immature red blood cells (reticulocytosis), and Macrocytic anemia and others. 27 total HPO annotations.

Organ System	Phenotype Count	Example Features
Blood and immune system	10

Prognosis

Phenotype severity distribution: 3 very common features, 10 common features.

Estimated prevalence: <1 in 1,000,000 (VERY_RARE).

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

1 clinical trial registered, 1 recruiting. Interventions under study include biologic therapy. Pipeline includes 1 NA. Research is primarily sponsored by academic and government institutions.

28 publications have been identified in PubMed for dehydrated hereditary stomatocytosis. Research spans Basic Science / Preclinical (43%), Case Report / Case Series (39%), and Review / Meta-Analysis (11%).

Research Type	Count	% of Total
Laboratory research	12

Data assembled from 5 of 12 sources · Last updated Sep 19, 2026, 2:54 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

Orphanet

European rare disease database

Orphanet:3202

GARD

Genetic and Rare Diseases Info Center

GARD:5623

Questions for Your Care Team

→Are any of the 1 active clinical trial a good fit for my situation?
→Are there specialists or centers of excellence for this condition in my area?
→Is there a patient registry I should consider joining?

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dehydrated hereditary stomatocytosis

Overview

Clinical Presentation

Prognosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

Questions for Your Care Team

Questions for Your Care Team

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Recent Publications

dehydrated hereditary stomatocytosis

Overview

Clinical Presentation

Prognosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

Subtypes

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

Explore clinical features

Recent Publications