Sickle Cell Anemia Treatment


Anemia - the private-year and the (ancient Greek haimos), "blood" - is an anomaly of the blood characterized by a decrease in the concentration of hemoglobin intra-cell and the lack of erythrocytes, or red blood cells or GR. This lack leads to a poor transport of oxygen through blood.

It is diagnosed by blood or numeration NFS examination carried out on a blood test.

If the decrease is red blood cells and thrombocytes or leukocytes, one speaks of bicytopenie. If all three lines are down, we talk pancytopenia.

The symptoms are common to several anaemia are:

* Asthenia (large fatigue);
* Muscular fatigue;
* Dyspnea, polypnea;
* Tachycardia, palpitations, (indeed, to maintain a proper oxygenation of the peripheral tissues with less GR (therefore low hemoglobin), the heart must accelerate its pace)
* Pallor (especially at conjonctives: look inside the lower eyelid);
* Headache;
* False dizziness;
* Lipothymie;
* Disorders dander: brittle hair, nails flattened.

In acute anaemia cause dizziness and a rapid heartbeat during intense efforts even slightly. On some land, it can be manifested by pain in the chest (angina). If anemia is chronic and very long (several years), one can even see appear ultimately a non-obstructive cardiomyopathy hypertrophy.

In cardiac auscultation, we can find a breath functional (ie without cardiac abnormality underlying). Indeed, the decline in the number of red blood cells in the blood leads to a decrease in blood viscosity. The laminar flow normally in the heart becomes turbulent and therefore created a heart murmur reversible after correction of anemia.

The anemia are classified into several families as the mechanism of anemia:

* Anemia central rate is decreased reticulocyte:
o myelosuppression
o anomaly in the structure of the bone marrow hematopoietic
o hormonal stimulation decreased (EPO)
o by iron deficiency anemia (iron deficiency anemia)
malabsorption of iron
occult bleeding (digestive) / abundant menstruation ( "chlorosis girls")
o deficiency anaemia by vitamin B12 or folate
o production of erythropoiesis inhibitor, as in inflammation.

* Anemia device: the rate of reticulocytes is normal or increased
o blood loss aigue
haemorrhages, including blood loss and repeated hidden in a colon cancer
o hemolytic anemia: destruction of red blood cells (hemolysis)
Because extra-Corpuscular:
# Immunological, auto-immune
# Hemolytic disease of the newborn
# Splenomegaly: spleen too big trap red blood cells
Corpuscular cause: the blood is destroyed because of its fragility
# Sickle cell disease (sickle cell anemia)
# Thalassaemia
o regeneration after anemia Central

They can also be classified according to their characteristics on the blood:

* Anemia aregenerative: reticulocytes are no longer products that sign anemia Central.
* Hypochromic anemia
* Macrocytic anemia: the size of red blood cells (VGM) exceeds the standard, vitamin B12 or folate, for example.
* Microcytic anemia: the size of red blood cells (VGM) is less than the standard, by iron deficiency, for example.

Exploration of anemia
It is important to mention that the number of red blood cells and haemoglobin levels are lower in women and children than among men (because of its androgenic impregnation).

1) The number of red blood cells is normally:

* 5 (4.5 to 5.5) million / mm ³ in humans adult;
* 4.5 (3.9 to 5.0) million / mm ³ female adult and child.

2) The haemoglobin (Hb) of blood is normally:

* 15 (13.5 to 16) g/100 mL in the adult male;
* 13.5 (11.5 to 15) g/100 mL in the adult woman and child.

There is talk of anemia if the hemoglobin is less than 13 g / dl in men and adults less than 12 g / dl in women.

Among pregnant women, there is a physiological hemodilution, then place the minimum rate at 11 g / dL.

Bilan biological
To diagnose anemia, only NFS (numerals and Formula globular Sanguine) is necessary. In addition, there will always mix reticulocytes. Indeed, on behalf of reticulocytes can determine if anemia is regenerative (there is increased birth of new GR to adapt, so the manufacturing process GR works well but there is increased loss explaining anemia) or aregenerative (there is no manufacture of new GR or at least not for adjusting the production (which should increase) the loss.

To determine the aetiology of anaemia, depending on the context, we can achieve different analyses by suspected cause:

* Review martial: serum iron, ferritin, total capacity of fixing the transferrin saturation of transferrin
* Vitamin B12 serum ± intra-erythrocyte
* Folates serum ± intra-erythrocyte
* Profile inflammatory protein
* Ultra-sensitive TSH, T4
* Bilirubin total and combined
* Lactate dehydrogenase (LDH)
* Haptoglobine
* Rubbing blood on his finger
* Search agglutinins irregular by direct Coombs test ± Coombs awareness
* Hemocultures
* Sérologies parasitic
* Rubbing blood thick and drop the search for malaria
* Electrophoresis of hemoglobin
* Punction ± medullary bone marrow biopsy with colour Perls
* Test resistance of red blood cells to hemolysis sucrose, or acidic
* Determination of G6PD and the pyruvate kinase
* Study the lifespan of red blood marked the technetium 99m
* Test Schilling
* Plombemie because anemia can be induced by lead poisoning
* Exploration of the hemostasis: TP, TCA, Time bleeding, clotting factors
* Creatinine, urea, uric acid

Special cases
* Aplasia by anemia is a rare type to unknown causes. It is characterized by the absence or decrease in erythroblastes (precursors of red blood cells) in bone marrow. It may also be due to leukemia.
* Sickle cell anemia: blood have a form stretched reducing their ability to transport oxygen. It is a mutation molecules of hemoglobin.
* Iron deficiency anaemia: The origin is a lack of iron fixer of oxygen.
* Anemia by paroxysmal nocturnal hemoglobinuria.

The principle of treating anemia is the treatment of its etiology. In some emergency situations where anaemia is symptomatic, even profound, a blood transfusion is performed.

EPO, or erythropoietin
This miracle has changed the lives of surgery scheduled for major surgery (column, knees, hips) reducing indications transfusion counterpart source of immunosuppression (eg knee). Its introduction in France late (too) in this indication (see Germany) is without doubt a scandal that remain less well known than the "contaminated blood."

Blood transfusion
It uses the concentrated cells from donated blood. They are infused with anemic respecting the compatibility of blood groups and phenotypes and rhesus.

Apart from an acute bleeding which does not require a minimum balance, no arguments etiological clinical asked before transfusion, not to distort research conducted later:

* Numerals, formula, plates with numbers of reticulocytes;
* Rh and ABO grouping Search agglutinins irregular (RAI);
* Serum iron multiplier saturation of the sidérophylline;
* Determination of folate and vitamin B12;
* Bilirubines full and free;
* Direct Coombs test.

The diagnosis of anemia stems from the knowledge of the mechanisms that underlie.

In a first time, data from blood (blood with a number of red blood cells, white blood cells and platelets + determination of hemoglobin and hematocrit with calculating the mean corpuscular volume (MCV) and the average content Corpuscular hemoglobin (TCMH: average grade of GR in Hb)) and the rate of reticulocytes guide the research.

Before a microcytic anemia, the dosage ferritin allows the distinction between martial deficiencies (iron deficiency) anemia and inflammatory. A hyperréticulocytose evokes hemolytic anemia, in which context it is worth looking first hemolytic anemia auto-immune.

The macrocytic anemia normocytaires or non-regenerative must seek a central cause by the myélogramme or osteo-bone marrow biopsy.

Read also Leukemia