Hemolysis in Patients With Hereditary Spherocytosis (HS)

Not Applicable

• Conditions: Hereditary; Hemolysis
• Interventions: Other: fermented papaya preparation (FPP)

• Registration Number: NCT01201174
• Lead Sponsor: Wolfson Medical Center

Brief Summary

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

Detailed 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 \[5\].

Study Timeline

• Status Verified: 2010-09
• Study Start: 2010-09
• Study First Submitted: 2010-09-12
• Study First Submitted QC: 2010-09-13
• Last Update Submitted: 2010-09-13
• Study First Posted: 2010-09-14
• Last Update Posted: 2010-09-14
• Primary Completion: 2011-09
• Study Completion: 2011-09

Recruitment & Eligibility

• Status: UNKNOWN
• Sex: All
• Target Recruitment: 15

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

Study & Design

• Study Type: INTERVENTIONAL
• Study Design: PARALLEL

Arm && Interventions

Group	Intervention	Description
Patients with Hereditary Spherocytosis	fermented papaya preparation (FPP)	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.

Primary Outcome Measures

Name	Time	Method
ROS, reduced glutathione (GSH) and lipid peroxides will be measured in RBC	year	ROS, reduced glutathione (GSH) and lipid peroxides will be measured in RBC following incubation with with 100 μM 2'-7'-dichlorofluorescin diacetate, 40 μM \[1-(4-chloromercuryphenyl-azo-2-naphthol)\] and fluor-DHPE, respectively for ROS \[8\] and with mercury orange for GSH \[9\]. After being washed twice, the cells will be resuspended in PBS and analyzed by flow cytometry .

Secondary Outcome Measures

Name	Time	Method
Hemolysis	year	Hemolysis will be assayed by suspending 3 ml of packed RBC in PBS or in the autologous plasma and overnight incubation in the presence of various concentrations of antioxidants such as fermented papaya preparation (FPP)

Trial Locations

Locations (1)

Wolfsson Medical Center; 🇮🇱 Holon,, Israel,, Israel