Caring for Clients with the Disorders of the Hematopoetic System

Anemia
From

the Greek word An-haimia, meaning no blood This results in a reduced ability of blood to transfer oxygen to the tissues, causing tissue hypoxia. Since all human cells depend on oxygen for survival, varying degrees of anemia can have a wide range of clinical consequences.

 Hemoglobin

(the oxygen-carrying protein in the red blood cells) has to be present to ensure adequate oxygenation of all tissues and organs.

 The

three main classes of anemia include excessive blood loss (acutely such as a hemorrhage Chronically through low-volume loss), excessive blood cell destruction (hemolysis) Deficient red blood cell production (ineffective hematopoiesis). In menstruating women, dietary iron deficiency is a common cause of deficient red blood cell production.

 Hemoglobin

(the oxygen-carrying protein in the red blood cells) has to be present to ensure adequate oxygenation of all tissues and organs. The three main classes of anemia include excessive blood loss (acutely such as a hemorrhage or chronically through low-volume loss), excessive blood cell destruction (hemolysis) or deficient red blood cell production (ineffective hematopoiesis). In menstruating women, dietary iron deficiency is a common cause of deficient red blood cell production.

Classification of Anemia
Pernicious

Anemia Aplastic Anemia Sickle Cell Anemia Iron Deficiency Anemia Cooleys Anemia

 Pernicious

anemia (also known as

Biermer's anaemia or Addison's anaemia or Addison-Biermer anaemia)

 is

a form of megaloblastic anaemia due to vitamin B12 deficiency dependent on impaired absorption of vitamin B12 in the setting of atrophic gastritis, and more specifically of loss of gastric parietal cells. The term "pernicious anaemia" is sometimes also incorrectly used to indicate megaloblastic anaemia due to any cause of vitamin B12 deficiency, its proper usage refers to that caused by atrophic gastritis and parietal cell loss only.

Pathophysiology

Vitamin B12 cannot be produced by the human body, and must therefore be obtained from diet. Normally, dietary vitamin B12 can only be absorbed by the ileum when it is bound by the intrinsic factor produced by parietal cells of the gastric mucosa. In pernicious anaemia, this process is impaired because of loss of parietal cells, resulting in insufficient absorption of the vitamin, which over a prolonged period of time ultimately leads to vitamin B12 deficiency and thus megaloblastic anaemia.

Diagnosis
A

diagnosis of pernicious anaemia first requires demonstration of megaloblastic anaemia (through a full blood count) and of its direct cause, vitamin B12 deficiency (by measuring B12 levels in serum). A Schillings test can then be used to distinguish pernicious anemia from other causes of vitamin B12 deficiency (notably malabsorption. A diagnosis of atrophic gastritis should be confirmed by gastroscopy with biopsies. Approximately 90% of individuals with pernicious anemia have antibodies for parietal cells, however only 50% of individuals with these antibodies have pernicious anaemia

Treatment
Being

a manifestation of vitamin B12 deficiency, pernicious anaemia is treated by administering vitamin B12 supplements. Note that if oral tablets are chosen for this purposes, much higher doses are given than normally required in order to overcome the impaired absorption that characterises pernicious anaemia. If oral tablets are not sufficient, vitamin B12 can also be administered via injection. Often the patient can learn to do this at home.

Nurses Resposibility
If

you are to administer Vit B Com, make sure to use Z-track technique/ deep IM Use different syringe/ aspirating needle to prevent skin stain especially for lighter people Route for injection is gluteal, avoiding the sciatic nerve Avoid vigorous activities Avoid smoking (depletes Vit B)

Pernicious Anemia

RBC Lacking in Vitamin B Complex

Aplastic Anemia

 Aplastic

anemia is a condition where bone marrow does not produce sufficient new cells to replenish blood cells. The term 'aplastic' means the marrow suffers from an aplasia that renders it unable to function properly. Anemia is the condition of having fewer red blood cells than normal, or fewer than needed to function properly. Typically, anemia refers to low red blood cell counts, but aplastic anemia patients have lower counts of all three blood cell types: red blood cells, white blood cells, and platelets.

Causes

One known cause is an autoimmune disorder, where the white blood cells attack the bone marrow. In many cases, the etiology is impossible to determine, but aplastic anemia is sometimes associated with exposure to substances such as benzene, radiation, or to the use of certain drugs, including chloramphenicol, carbamazepine, phenytoin, quinine, and phenylbutazone. Many drugs are associated with aplasia mainly in the base of case reports but at a very low probability, As an example, chloramphenicol treatment is followed by aplasia in less than 1 in 40,000 treatment courses,and carbamazepine aplasia is even more rare. Aplastic anaemia is present in up to 2% of patients with acute viral hepatitis.

Signs and symptoms

Anemia with malaise, pallor and associated symptoms Thrombocytopenia (low platelet counts), leading to increased risk of hemorrhage and bruising Leukopenia (low white blood cell count), leading to increased risk of infection

Diagnosis

The diagnosis can only be made on bone marrow examination. Before this procedure is undertaken, a patient will generally have had other blood tests to find diagnostic clues, including a full blood count, renal function and electrolytes, liver enzymes, thyroid function tests, vitamin B12 and folic acid levels.

Treatment

Treating aplastic anemia involves suppression of the immune system, an effect achieved by daily medicine intake, or, in more severe cases, a bone marrow transplant, a potential cure but a risky procedure. The transplanted bone marrow replaces the failing bone marrow cells with new ones from a matching donor. The pluripotent stem cells in the bone marrow reconstitute all three blood cell lines, giving the patient a new immune system, red blood cells, and platelets. However, besides the risk of graft failure, there is also a risk that the newly created white blood cells may attack the rest of the body ("graft-versus-host disease"). Medical therapy of aplastic anemia often includes a short course of anti-thymocyte globulin (ATG or antilymphocyte globulin) and several months of treatment with cyclosporin to modulate the immune system. Mild chemotherapy with agents such as cyclophosphamide and vincristine may also be effective.

Nursing Responsibility
Use

soft bristle toothbrush Avoid sharp pointed objects Avoid ADL that involves gardening, sewing and cooking Promote rest and avoidance of stress (triggers/ aggravates the s/s) Avoid taking ASA

Sickle Cell Disease

Sickle-cell

disease (SS) is a group of genetic disorders caused by sickle haemoglobin (Hgb S or Hb S). In many forms of the disease, the red blood cells change shape upon deoxygenation because of polymerization of the abnormal sickle hemoglobin; the hemoglobin proteins stick to each other, causing the cell to get a rigid surface and sickle shape.

 This

process damages the red blood cell membrane, and can cause the cells to become stuck in blood vessels. This deprives the downstream tissues of oxygen and causes ischemia and infarction, which may cause organ damage, such as stroke. The disease is chronic and lifelong. Individuals are most often well, but their lives are punctuated by periodic painful attacks.

Signs and symptoms

Vaso-occlusive crises A vaso-occlusive crisis is caused by sickle-shaped red blood cells that obstruct capillaries and restrict blood flow to an organ, resulting in ischemia, pain, and organ damage. Because of its narrow vessels and function in clearing defective red blood cells, the spleen is frequently affected. It is usually infarcted before the end of childhood in individuals suffering from sickle-cell anemia. This autosplenectomy increases the risk of infection from encapsulated organisms; Bones, especially weight-bearing bones, are also a common target of vaso-occlusive damage. This is due to bone ischemia. A recognized type of sickle crisis is the acute chest crisis, a condition characterised by fever, chest pain, hard breathing, and pulmonary infiltrate on chest X-ray. Given that pneumonia and intrapulmonary sickling can both produce these symptoms, the patient is treated for both conditions.

Other sickle-cell crises Aplastic crises are acute worsenings of the patient's baseline anaemia producing pallor, tachycardia, and fatigue. This crisis is triggered by parvovirus B19, which directly affects erythropoiesis (production of red blood cells). Parvovirus infection nearly completely prevents red blood cell production for 2-3 days. In normal individuals this is of little consequence but the shortened red cell life of sickle-cell patients results in an abrupt, life-threatening situation. Reticulocyte counts drop dramatically during the illness and the rapid turnover of red cells leads to the drop in hemoglobin. Most patients can be managed supportively; some need blood transfusion. Splenic sequestration crises are acute, painful enlargements of the spleen. The abdomen becomes bloated and very hard. Management is supportive, sometimes with blood transfusion.

Complications of Sickling
Severe

infection Priapism Acute Coronary Syndrome Stroke Cholecystitis Peripheral Arterial Obstructive Disease

Thalassemia (Cooleys Anmeia)

Thalassemia

(British spelling, "thalassaemia") is an inherited autosomal recessive blood disease. In thalassemia, the genetic defect results in reduced rate of synthesis of one of the globin chains that make up hemoglobin. Reduced synthesis of one of the globin chains causes the formation of abnormal hemoglobin molecules, and this in turn causes the anemia which is the characteristic presenting symptom of the thalassemias.

Cooleys Anemia

 There

is no cure for thalassemias, and the best treatment available today consists of frequent blood transfusions (every two to three weeks) with iron chelation therapy (e.g. deferoxamine) administered subcutaneously. Bone marrow transplants (hematopoietic stem cell transplantations) and cord blood transplantation with preoperative myeloablation are potentially curative, though the latter requires further investigation.

The thalassemias are classified according to which chain of the hemoglobin molecule is affected (see hemoglobin for a description of the chains). In thalassemias, production of the globin chain is affected, while in thalassemia production of the globin chain is affected. Thalassemia produces a deficiency of or globin, unlike sickle-cell disease which produces a specific mutant form of globin. globin chains are encoded by a single gene on chromosome 11; globin chains are encoded by two closely linked genes on chromosome 16. Thus in a normal person with two copies of each chromosome, there are two loci encoding the chain, and four loci encoding the chain. Deletion of one of the loci has a high prevalence in people of African-American or Asian descent, making them more likely to develop thalassemias. thalassemias are common in African-Americans, but also in Greeks and Italians.

Sickle Cell Anemia

Nursing Management for SCA

H-

Hydration O- Oxygen R- Rest A- Analgesic F Fluids E- Elevation

Polycythemia Vera

Polycythemia vera is an abnormal increase in blood cells (primarily red blood cells) due to excess production of the cells by the bone marrow.

Causes Polycythemia vera is an acquired disorder of the bone marrow that causes the overproduction of white blood cells, red blood cells, and platelets. It is a rare disease that occurs more frequently in men than women, and rarely in patients under age 40. The exact cause is unknown.

Symptoms
Breathing Dizziness Fullness

difficulty when lying down

in the left upper abdomen Headache Itchiness, especially following a warm bath Red coloration, especially of the face Shortness of breath Symptoms of phlebitis

Additional symptoms that may be associated with this disease:

Bluish

skin discoloration Fatigue Skin spots, red Vision problems

Exams and Tests

The

health care provider will perform a physical exam. Tests that may be done include: Bone marrow biopsy Blood volume Chemistry panel Complete blood count with differential Hematocrit White blood count Platelet count Vitamin B12 level

Treatment

The goal of treatment is to reduce the thickness of the blood and to prevent bleeding and clotting. A method called phlebotomy is used to decrease blood thickness. One pint of blood is removed weekly until the hematocrit level is less than 45, then therapy is continued as needed. Occasionally, chemotherapy (specifically hydroxyurea) may be given to suppress the bone marrow. Interferon may be given to lower blood counts. A medicine called anegrelide may be given to lower platelet counts. The use of blood thinners (such as aspirin) is controversial because it may cause stomach bleeding.

Outlook (Prognosis)
The

disease usually develops slowly, and most patients do not experience any problems related to the disease after being diagnosed. However, the abnormal bone marrow cells may begin to grow uncontrollably in some patients leading to acute myelogenous leukemia. This occurs in a minority of cases. Patients with polycythemia vera also have an increased tendency to form blood clots that can result in strokes or heart attacks. Some patients may experience abnormal bleeding because their platelets are abnormal.

Possible Complications
Gastric

bleeding

Gout
Heart

failure Leukemia Myelofibrosis Peptic ulcer disease Thrombosis (a cause of stroke or heart attack)