Hemolysis in Patients With Hereditary Spherocytosis (HS)

Wolfson Medical Center (WMC)

Status: Unknown

Conditions: Hemolysis; Hereditary

Treatments: Other: fermented papaya preparation (FPP)

Study type: Interventional

Funder types: Other

Identifiers

NCT01201174

SH-01CTIL

Details and patient eligibility

About

In the present study the investigators are going to explore the oxidative status of HS-RBC and its contribution to hemolysis

Full description

The oxidative status of cells, which is determined by the balance between pro-oxidants, such as the reactive oxygen species (ROS), and antioxidants, is a major regulator of cellular functions. Impaired balance between pro- and antioxidants causes oxidative stress which may result in oxidation of proteins, lipids and DNA with the final outcome of premature cell aging and apoptosis [1,2]. Oxidatively stressed red blood cell (RBC) have been observed in various congenital and acquired hemolytic anemias, including thalassemia, sickle cell anemia, congenital dyserythropoietic anemia, G6PD deficiency and paroxysmal nocturnal hemoglobinuria (PNH) as well as in myelodysplastic syndrome (MDS). Although the primary etiology is different in these anemias, oxidative stress mediates several of their pathologies, mainly hemolysis [3].

Hereditary Spherocytosis (HS) is a genetic disorder of the RBC skeleton with primary deficiency in spectrin, ankyrin-1, band 3 or protein 4.2 associated with chronic hemolytic anemia [4]. Secondary protein deficiencies resulting from oxidative stress are often observed and may be involved in the clinical manifestations of the disease [5].

Enrollment: 15 estimated patients

Sex: All

Ages: 5+ years old

Volunteers: Accepts Healthy Volunteers

Inclusion criteria

• patients > 5 years
• with documented family history of HS
• patients should have clinical and laboratory findings, consistent with mild to severe HS, diagnosed on the basis of spherocyte morphology, elevated MCHC (33-38 g/dl), with a mean value of (35.47 g/dl), increased osmotic fragility , splenomegaly and non-immune mediated hemolysis.

Exclusion criteria

• non

Trial design

Primary purpose: Diagnostic

Allocation: Non-Randomized

Interventional model: Parallel Assignment

Masking: None (Open label)

---

15 participants in 1 patient group

Patients with Hereditary Spherocytosis

No Intervention group

Description:

All patients should have clinical and laboratory findings, consistent with mild to severe HS, diagnosed on the basis of spherocyte morphology, elevated MCHC (33-38 g/dl), with a mean value of (35.47 g/dl), increased osmotic fragility , splenomegaly and non-immune mediated hemolysis.

Treatment:

Other: fermented papaya preparation (FPP)