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The Gallstone Elimination Report

Gallstone Natural Solutions by David Smith

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Symptoms

None

None

Pallor, erythroblastopenic crises, splenomegaly, gallstones

Pallor, erythroblastop crises, splenomegal gallstones

"Value before transfusion.

•"Normal (mean±SD): 226 ± 54 x 103 molecules per cell. From Eber SW, Armburst R, Schröter W. J Pediat 1990;117:409.

"Value before transfusion.

•"Normal (mean±SD): 226 ± 54 x 103 molecules per cell. From Eber SW, Armburst R, Schröter W. J Pediat 1990;117:409.

4. Gallstones: In approximately one-half of untreated patients; increased incidence with age. Occasionally, HS may be masked or improved in obstructive jaundice due to increase in surface area of red cells and formation of targets cells

5. Hemochromatosis: Rarely.

Treatment

1. Folic acid supplement (1 mg/day)

2. Leukocyte-depleted packed red cell transfusion for severe erythroblastopenic crisis

3. Splenectomy* for moderate to severe cases. Most patients with less than 80% of normal spectrin content require splenectomy. Splenectomy should be carried out early in severe cases but not before 5 years of age, if possible. The management of the splenectomized patient is detailed in Chapter 26. Although sphero-cytosis persists postsplenectomy, the red cell life span becomes essentially normal and complications are prevented, especially transient erythroblastope-nia and persistent hyperbilirubinemia, which leads to gallstones

4. Ultrasound should be carried out before splenectomy to exclude the presence of gallstones. If present, cholecystectomy is also indicated.

Hereditary Elliptocytosis

Hereditary elliptocytosis (HE) is clinically and genetically a heterogeneous disorder. Pathogenesis

HE is due to various defects in the skeletal proteins, spectrin, and protein 4.1. The basic membrane defects consist of:

1. Defects of spectrin self-association involving the a-chains

2. Defects of spectrin self-association involving the P-chains

3. Deficiency of protein 4.1

4. Deficiency of glycophorin.

Deficiencies of these skeletal proteins result in decreased horizontal stability and reduced pliability of red blood cells. Thus, a red blood cell is unable to regain its biconcave shape after its distortion in the microcirculation.

The membrane defect results in decreased cellular deformability as a result of increased membrane rigidity, which is a consistent feature in all cases. In addition, cell dehydration is present. In HE, cell fragmentation is the result of the loss of mechanical integrity of the skeleton. The cell remnants contain a full complement of various membrane proteins and represent a true fragmentation process in which microcytic red blood cells with decreased hemoglobin content are generated. In HS, however, continuous loss of the lipid-rich and skeleton-free domains of the membrane during the red cell life span results in spherocytic red cells with near-normal hemoglobin content and the absence of fragmented cells with markedly decreased hemoglobin content.

*Laparoscopic splenectomy is safe in children. Although it requires more operative time than open splenectomy, it is superior with regard to postoperative analgesia, smaller abdominal wall scars, duration of hospital stay, and more rapid return to a regular diet and daily activities. It is not known if accessory spleens are readily identified with the laparoscope although the magnification afforded by the laparo-scope might be advantageous in some cases.

Genetics

HE is characterized by an autosomal dominant mode of inheritance (with variable penetrance), affecting about 1 in 25,000 of the population. Two types of inheritance occur:

1. Non-Rh-linked, associated with a high incidence of severe anemia in the homozygote

2. Rh-linked, usually associated with a milder disorder. Clinical Features

1. Varies from patients who are symptom free to severe anemia requiring blood transfusions. The percentage of microcytes best reflects the severity of the disease.

2. About 12% have symptoms indistinguishable from hereditary spherocytosis.

3. The percentage of elliptocytes varies from 50% to 90%. No correlation has been established between the degree of elliptocytosis and the severity of the anemia.

4. HE has been classified into the following clinical subtypes:

a. Common HE, which is divided into several groups: silent carrier state, mild HE, HE with infantile pyknocytosis b. Common HE with chronic hemolysis, which is divided into two groups: HE with dyserythropoiesis and homozygous common HE, which is clinically indistinguishable from hereditary pyropoikilocytosis (see later discussion)

c. Spherocytic HE, which clinically resembles HS; however, a family member usually has evidence of HE

d. Southeast Asian ovalocytosis, in which the majority of cells are oval; however, some red cells contain either a longitudinal or transverse ridge.

Laboratory Findings

1. Blood smear: 25-90% of cells elongated oval elliptocytes

2. Osmotic fragility normal or increased

3. Autohemolysis usually normal but may be increased and usually corrected by the addition of glucose or ATP.

Treatment

The indications for transfusion, splenectomy, and prophylactic folic acid are the same as for hereditary spherocytosis.

Hereditary Pyropoikilocytosis

Definition

Hereditary pyropoikilocytosis (HPP) is a congenital hemolytic anemia associated with in vivo red cell fragmentation and marked in vitro fragmentation of red cells at 45°C. Because of the similarities in the membrane defect in this condition and HE, it is viewed as a subtype of HE.

Genetics and Etiology

1. Homozygous or doubly heterozygous for the spectrin chains (e.g., Sp-a1/74 and Sp-a1/46). The spectrin chain defects found in HPP are similar to those found in HE.

2. Increased ratio of cholesterol to membrane protein.

3. Decreased cell deformability.

Clinical Features

1. Anemia characterized by extreme anisocytosis and poikilocytosis a. Red cell fragments, spherocytes, and budding red cells (the red cells are exquisitely sensitive to temperature and fragment after 10 minutes of incubation time at 45-46°C in vitro; heating for 6 hours at 37°C explains in vivo formation of fragmented red cells and chronic hemolysis)

b. Hemoglobin level, 7-9 g/dL

c. Marked reduction in MCV and elevated MCHC

2. Jaundice

3. Splenomegaly

4. Osmotic fragility and autohemolysis increased

5. Mild HE present in one of the parents or siblings.

Differential Diagnosis

Similar cells are seen in microangiopathic hemolytic anemias, after severe burns or oxidant stress, and in pyruvate kinase deficiency.

Treatment

Patients respond well to splenectomy with a rise in hemoglobin to 12 g/dL. Following splenectomy, hemolysis is decreased but not totally eliminated.

Hereditary Stomatocytosis Definition and Genetics

The stomatocyte has a linear slit-like area of central pallor rather than a circular area. When suspended in plasma, the cells assume a bowl-shaped form. This hereditary hemolytic anemia of variable severity is characterized by an autosomal dominant mode of inheritance.

Etiology

The cells contain high Na+ and low K+ concentrations. The disorder is probably due to a membrane and protein defect. The cells are abnormally rigid and poorly deformable, contributing to their rapid rate of destruction. There are many biochemical variants.

Clinical Features

1. Very variable

2. Jaundice at birth

3. Pallor: marked variability depending on severity of anemia

4. Splenomegaly

5. Hematology a. Anemia b. Smear, 10-50% stomatocytes c. Reticulocytosis d. Increased osmotic fragility and autohemolysis.

Differential Diagnosis

Stomatocytosis may occur with thalassemia, some red cell enzyme defects (glu-tathione peroxidase deficiency, glucose phosphate isomerase deficiency), Rhnull red cells, viral infections, lead poisoning, some drugs (e.g., quinidine and chlorpro-mazine), some malignancies, liver disease, and alcoholism.

Treatment

Splenectomy may be beneficial if hemolysis is severe.

Hereditary Acanthocytosis

Definition

Acanthocytes have thorn-like projections that vary in length and width and are irregularly distributed over the surface of red cells.

Genetics

The mode of inheritance is autosomal recessive.

Clinical Features

1. Steatorrhea: Only fat malabsorption

2. Neurologic symptoms: Weakness, ataxia and nystagmus, atypical retinitis pigmentosa with macular atrophy, blindness

3. Anemia: Mild hemolytic anemia; 70-80% acanthocytes; slight reticulocytosis.

Diagnosis

1. Clinical syndrome

2. Absent b-lipoprotein in plasma

3. Diagnostic findings on small intestine biopsy.

Differential Diagnosis

During the neonatal period, hereditary acanthocytosis may have to be distinguished from the benign nonhereditary disorder of infantile pyknocytosis. Acquired acan-thocytosis occurs under the following conditions: renal failure, cirrhosis, microangio-pathic hemolytic anemia, hypothyroidism, pyruvate kinase deficiency, and in association with some neoplasms.

Hereditary Xerocytosis

Definition

Hereditary xerocytosis is a familial condition characterized by red cells that appear to be shrunken, with hemoglobin puddled at the periphery or center of the cell. The defect in these cells permits increased permeability of univalent cations Na+ and K+. The accompanying cell water loss results in dehydrated red cells. There is an increased proportion of phosphatidylcholine in the membrane.

Genetics

The mode of inheritance of this rare condition is autosomal dominant.

Clinical Features

1. Few symptoms, moderate anemia; red cell morphology, stomatocytic

2. Elevated reticulocytes

3. Splenomegaly and gallstones

4. MCHC elevated, MCV increased

5. Osmotic fragility reduced

6. Increased heat stability (46 and 49°C for 60 minutes).

Treatment

Transfusions are generally not required. The benefit of splenectomy is slight.

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Get Rid of Gallstones Naturally

Get Rid of Gallstones Naturally

One of the main home remedies that you need to follow to prevent gallstones is a healthy lifestyle. You need to maintain a healthy body weight to prevent gallstones. The following are the best home remedies that will help you to treat and prevent gallstones.

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