5Th Quarter Heme Part 4

Front 1

In these anemia the erythrocytes have a shortened life span. Thus their number in the circulation is reduced. Fewer erythrocytes result in decreased available oxygen, causing hypoxia which in turn stimulates and increase in erythropoietin release from the kidney. This then stimulates the bone marrow to compensate by producing new erythrocytes and releasing some of them into circulation somewhat prematurely as reticulocytes

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Hemolytic anemias

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Name two inherited forms of hemolytic anemia

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Sickle cell anemia and thalassemia

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This anemia is a severe hemolytic anemia that results from inheritance of the sickle hemoglobin HBS Gene which causes the hemoglobin molecule to be defective

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Sickle cell anemia

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What is the Sickle Cell hemoglobin Gene

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HBS

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HBS acquires a crystal like formation when exposed to low oxygen tension. The oxygen level in venous blood can be low enough to cause this change, consequently the erythrocyte containing HBS loses its round pliable, bioconcave disc shape and becomes dehydrated, rigid and sickle-shaped

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Sickle cell anemia

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These long rigid erythrocytes can adhere to the endothelium of small vessels, when they adhere to each other blood flow to a region or an organ may be reduced.

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Sickle cell anemia

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Ischemia or infarction may result from these adhering to each other and the patient may have pain, swelling and fever

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Sickle cell anemia

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The sickling process takes time, if the erythrocyte is again exposed to adequate amounts of oxygen before the membrane becomes too rigid it can revert to a normal shape. For this reason the sickling crisis is are intermittent

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Sickle cell anemia

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Cold can aggravate this process because vasoconstriction slows the blood flow.

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Sickling process, sickle cell anemia

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Oxygen delivery can also be impaired by an increased blood viscosity with or without occlusion due to adhesion of Sickle cells, in this situation the effects are seen in larger vessels such as arterioles

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Sickle cell anemia

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This Gene is inherited in people of African descent and a lesser extent in people from the Middle East, the Mediterranean and Aboriginal tribes in India

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HBS Gene, sickle cell anemia

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If two people with sickle cell trait have children the children may inherit two abnormal genes and will have sickle cell anemia

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True

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This type of anemia is diagnosed in infancy or early childhood, is incurable

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Sickle cell anemia

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Pain is a big problem with this anemia

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Sickle cell anemia

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Symptoms with this anemia result from chronic hemolysis or thrombosis.

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Sickle cell anemia

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Sickle cells are rapidly hemolyzed and thus have a very short lifespan of about this long

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10 to 20 days

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In sickle cell anemia the anemia is always present, usually hemoglobin values range between these levels

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7 and 10

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Jaundice is characteristic of this anemia and is usually obvious in the sclera.

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Sickle cell anemia

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The bone marrow expands in childhood in a compensatory effort to offset this anemia, sometimes leading to enlargement of the bones of the face and skull

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Sickle cell anemia

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Patients with this anemia are unusually susceptible to infection, particularly pneumonia and osteomyelitis

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Sickle cell anemia

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What are the three types of sickle cell crisis

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Acute vaso-occlusive crisis

Aplastic crisis

Sequestration crisis

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Results from entrapment of erythrocytes and leukocytes in the micro circulation which causes tissue hypoxia, inflammation and necrosis due to inadequate blood flow. When perfusion is resumed substances such as free radicals are released which leads to oxidative damage to the vessel. Thus the endothelium of the vessel becomes dysfunctional

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Acute vaso-occlusive crisis, sickle cell anemia

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Results from infection with human parvovirus. The hemoglobin level Falls rapidly and the marrow cannot compensate as evidenced by absent reticulocytes

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Aplastic crisis, sickle cell anemia

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Results when other organs pool the sickled cells. Although the spleen is the most common organ responsible for this in children, most children with sickle cell anemia have a splenic infarction by 10 years of age and the spleen is then no longer functional. In adults the common organs involved in this are the liver and more seriously the lungs

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Sequestration crisis, sickle cell anemia

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When not in Sickle Cell crisis patients with this anemia have many other symptoms

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False, most often with sickle cell anemia patients are asymptomatic except when in crisis

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Jaundice, pallor, fatigue, pale mucous membranes

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Associated with sickle cell anemia

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When in sickle cell crisis the primary symptom is this

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Severe pain

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Associated with aching of hands and feet as well as swelling of hands and feet

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Sickle Cell crisis

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Prolonged erection of the penis that lasts hours longer than or isn't even stimulated by sexual manner

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Priapism, associated with sickle cell crisis

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What triggers a Sickle Cell crisis

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Low blood levels of oxygen

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7 possible causes of sickle cell crisis

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Infection weather viral or bacterial

High altitude

Stress

Surgery

Blood loss

Dehydration

Acidosis

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List three cardiac complications of sickle cell anemia

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Cor pulmonale

Acute chest syndrome

CHF, enlarged heart, MI, heart failure

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Manifested by fever, respiratory distress and new infiltrates seen on x-rays. 75% of patients who develop this had a painful vaso-occlusive Sickle Cell crisis prior

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Acute chest syndrome

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List two respiratory complications of sickle cell anemia

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Pulmonary hypertension, infarction

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This is a common sequelae of sickle cell disease and often the cause of death

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Pulmonary hypertension

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List 4 other complications of sickle cell anemia

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Renal failure

Blindness

Thrombosis and stroke

Bone changes, leg ulcers

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Patients with this anemia are usually diagnosed in childhood because they become anemic in infancy and begin to have crisis is at 1 or 2 years of age. Some children die in the first years of life typically due to infection

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Sickle cell anemia

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Despite current management strategies the average life expectancy is still suboptimal typically in the fifth decade

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Sickle cell anemia

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The diagnosis of this condition is confirmed by hemoglobin electrophoresis

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Sickle cell anemia

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A chemotherapy agent that is effective in increasing fetal hemoglobin levels in patients with this condition. Thereby it decreases the formation of Sickle cells

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Hydroxyurea, for sickle cell anemia

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Patients who received this medication appear to have fewer painful episodes of crisis, a lower incidence of acute chest syndrome, and less need for transfusions

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Hydroxyurea for sickle cell anemia

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Side effects of this medication include chronic suppression of leukocyte formation, teratogenesis, potential for later development of malignancy.

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Hydroxyurea for sickle cell anemia

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4 treatments for sickle cell anemia

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Hydroxyurea

Long-term blood transfusions

Bone marrow or stem cell transplant

Supportive

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Pain is a significant issue with this condition

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Sickle cell anemia

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Adequate hydration is very important during a painful sickling episode.

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True

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Severe pain and fatigue are important considerations for healthcare providers in care of these patients

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Sickle cell anemia

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Acute pain during Sickle Cell crisis can be severe and unpredictable. Any joint that is acutely swollen should be supported and elevated until the swelling diminishes

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True

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This is a group of hereditary anemias characterized by hypochromia, extreme microcytosis, hemolysis, and variable degrees of anemia

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Thalassemia

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An abnormal decrease in the hemoglobin content of erythrocytes

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Hypochromia

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Smaller than normal erythrocytes

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Microcytosis

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This congenital anemia is characterized byy hypochromia, microcytosis and defective synthesis of hemoglobin

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Thalassemia

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Anemia associated with defective synthesis of hemoglobin, the production of one or more globulin chains within the hemoglobin molecule is reduced. This ultimately increases the rigidity of the erythrocytes and that's causes premature destruction of the cells

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Thalassemia

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These anemias are classified into two major groups According to which hemoglobin chain is diminished either alpha or beta

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Thalassemia

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The beta-thalassemia are milder than the alpha forms and often occur without symptoms

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False, the alpha thalassemia are milder than beta and often occur without symptoms

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Hsct offers a chance of cure but when this is not possible the most common treatment for this disease is transfusion of prbcs

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Thalassemia

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With this condition organ dysfunction due to iron overload results from excessive amounts of iron in multiple prbc transfusions. Chelation therapy can reduce the complications of iron overload and prolong the life of these patients

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Thalassemia

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Normally production of specific blood cells from their stem cell precursors is carefully regulated according to the body's needs. If the mechanisms that control the production of cells are disrupted the cells can proliferate excessively as seen in the development of hematologic neoplasms

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True

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Hematopoietic malignancies are often classified by the cells involved.

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True

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Refers to an increased level of leukocytes in the circulation

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Leukocytosis

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Typically only one specific cell type is increased in this condition

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Leukemia

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Although leukocytosis can be a normal response to increased need such as with infection the elevation in leukocytes should be decreased as the physiological needs decreases. Prolonged or progressively increasing elevation in leukocytes is abnormal and should be evaluated

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Leukemia

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This represents a group of malignant disorders that affect the blood, bone marrow, lymph system and spleen

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Leukemia

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The common feature of these conditions is unregulated proliferation of leukocytes in the bone marrow

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Leukemia

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Uncontrolled cell division and increase in leukocytes

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Leukemia

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Leukemia's are commonly classified according to the stem cell line involved either of these two varieties

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Lymphoid, stem cells producing lymphocytes

Myeloid, stem cells producing non lymphoid blood cells

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Leukemia's are classified according to Stem Cell line involved as well as either acute or chronic based on the time it takes for symptoms to evolve and the phase of cell development that is halted

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True

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This form of leukemia the symptom onset is abrupt often occurring within weeks.

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Acute leukemia

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Leukemia in which leukocyte development is halted at the blast phase in the most leukocytes are undifferentiated cells or blasts.

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Acute leukemia

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This leukemia can progressed rapidly with death occurring within weeks to months without aggressive treatment

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Acute leukemia

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In this leukemia symptoms evolve over a period of months to years

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Chronic leukemia

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In this leukemia the majority of leukocytes produced are mature

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Chronic leukemia

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This leukemia progresses more slowly, the disease trajectory can extend for years

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Chronic leukemia

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Although the exact cause of this condition is not known factors such as genetics, chromosomal changes, environment, chemical / radiation / chemotherapy exposure, viral may be attributed

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Leukemia

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Leukemia's tend to Metastasize to

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The brain

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Bone marrow aspiration for this condition would show lots of cells especially immature blood cells also known as blasts.

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Leukemia

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This percentage of the bone marrow cells are immature to diagnose leukemia

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Greater than 20%

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This leukemia results from a defect in the hematopoietic stem cell that differentiates into all myeloid cells such as monocytes, granulocytes, erythrocytes and platelets

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Acute myeloid leukemia

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This leukemia can occur at any age although it in frequently occurs before 55 years and the incidence Rises with age

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Acute myeloid leukemia

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This leukemia is the most common non lymphocytic leukemia

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Acute myeloid leukemia

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Age plays a significant role in prognosis for this leukemia. Younger patients May survive for 5 years or more after diagnosis. Patients older than 60 have a more undifferentiated form and poor prognosis

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Acute myeloid leukemia

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Secondary AML tends to be more resistant to treatment, resulting in a much shorter duration of remission. Even with treatment patients with secondary AML survive an average of less than one year with death usually resulting from infection or hemorrhage

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Acute myeloid leukemia

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This leukemia arises from a mutation in the myeloid stem cells. Normal myeloid cells continue to be produced but there is a pathologic increase in the production of forming of blast cells. So there will be a wide variety of cell types existing in the blood from blasts to mature cells

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Chronic myeloid leukemia

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This leukemia is uncommon in people younger than 20 years of age the incidence increasing with age and having a mean age of 65 years.

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Chronic myeloid leukemia

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Do to marked advances in treatment patients diagnosed with this leukemia in The Chronic phase have an overall median life expectancy well exceeding 5 years.

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Chronic myeloid leukemia

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During The Chronic phase of this leukemia patients have few symptoms and complications from the disease itself and problems with infection and bleeding are rare. However if the disease transforms to the acute phase, the disease becomes more difficult to treat

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Chronic myeloid leukemia

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This results from an uncontrolled proliferation of immature cells called lymphoblasts derived from the lymphoid stem cell

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Acute lymphocytic leukemia

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This leukemia is most common in young children with boys affected more often than girls. Peak incidence is 4 years of age. After 15 years of age it is relatively uncommon until age 50 when incidence rises again

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Acute lymphocytic leukemia

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This leukemia is very responsive to treatment, complete remission rates are approximately 85% for adults. Increasing age appears to be associated with diminished survival however

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Acute lymphocytic leukemia

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This leukemia is a common malignancy of older adults with average age of diagnosis at 72 years

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Chronic lymphocytic leukemia

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This is the most common form of leukemia in the United States and Europe

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Chronic lymphocytic leukemia

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A family history of this condition may be the most important risk factor for developing the condition

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Chronic lymphocytic leukemia

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Veterans of the Vietnam War who were exposed to agent orange may be at risk for developing this disease but there is no definitive link to other pesticides or chemical exposures.

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Chronic lymphocytic leukemia

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This type of leukemia is uncommon affecting only 2% of the population. Males greater than 40 years of age are at the highest risk

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Hairy cell leukemia

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No evidence by physical exam, and Diagnostics all appear normal

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Complete remission, leukemia

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Physical examination and some Diagnostics appear normal however bone marrow biopsy shows immature cells

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Partial remission, leukemia

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These two therapies are often used in combination in treatment of leukemia

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Chemotherapy and radiation

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List 4 treatments for leukemia

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Chemotherapy

Radiation

Bone marrow and stem cell transplantation

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Five major goals for leukemia in nursing

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Absence of complications

Pain control

Adequate nutrition (strong to fight disease)

Activity tolerance (increase as tolerated)

Self care (picc lines etc)

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With this condition although emphasis is placed on the oral mucosa the entire GI mucosa can be altered, not only by the effects of chemotherapy but also from prolonged administration of antibiotics

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Leukemia

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Nutritional intake is often reduced because of pain and discomfort associated with stomatitis. Encouraging or providing mouth care before and after meals in administering analgesic agents before eating can help increase intake.

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Leukemia

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Small frequent feedings of foods that are soft in texture and moderate in temperature may be better tolerated. Low microbial diets may be prescribed.

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Leukemia

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Fatigue is a common and oppressive symptom with this condition, nursing interventions should focus on assisting the patient to establish a balance between activity and

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Leukemia

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These patients should be assessed for signs of dehydration as well as fluid overload, with particular attention to pulmonary status and the development of dependent edema.

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Leukemia

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A group of clonal disorders of the myeloid stem cell that cause dysplasia (abnormal development) of one or more types of cell lines

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Myelodysplastic syndromes, MDS

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The most common feature of this condition is dysplasia of the erythrocytes, however the leukocytes and platelets can also be affected

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Myelodysplastic syndrome, MDS

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In this condition cells do not function normally. Neutrophils have diminished phagocytic ability, platelets are less able to aggregate.

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Myelodysplastic syndromes, MDS

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Although with this condition the bone marrow is actually hypercellular, many of the cells within it died before being released into circulation. Therefore the actual number of cells in the circulation is typically lower than normal

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Myelodysplastic syndromes, MDS

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The result of cellular defects and functions in this condition leads to increased risk of infection and bleeding even when the actual number of cells may not be excessively

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Myelodysplastic syndromes, MDS

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This form of myelodysplastic syndrome tends to be a disease of people older than 70 years. Because findings are subtle the diesease is not often diagnosed until later in the illness if at all. It may progress to AML

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Primary MDS

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This form of MDS can occur at any age and results from prior toxic exposure to chemicals such as chemotherapy agents.

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Secondary MDS

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This form of MDS has a poor prognosis than the other form

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Secondary MDS

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This form of MDS has a poor prognosis as it tends to be resistant to treatment, has more cytogenetic abnormalities and evolves into AML more frequently

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Secondary MDS

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many patients with this condition are asymptomatic with the illness being discovered incidentally when a CBC is performed for other purposes

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MDS

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Fatigue is often present and neutrophil dysfunction puts the person at risk for recurrent pneumonia and other infections

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MDS

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Due to platelet function alteration bleeding may occur. Overtime the marrow may fail to provide enough cells that can be supported with transfusion or growth factors this is called bone marrow failure

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MDS

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This condition may also progress over time as the dysplasia evolves into a leukemic state

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MDS

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Official diagnosis of this condition is based on the results of bone marrow aspiration and biopsy

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MDS

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CBC typically reveals a macrocytic anemia

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MDS

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What is hsct?

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Hematopoietic stem cell transplantation

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This is the only cure for MDS but is not often a viable option for most patients due to the presence of comorbidity or age

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Hsct

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Two medications considered in management of MDS

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Erythroid stimulating medications, procrit / epogen / aranesp

Gcsf medications, neupogen

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Chemotherapy is not always effective in the treatment of MDS

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True

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Transfusion of platelets and packed red blood cells may be required for patients with MDS. However it is important to watch for this

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Iron overload

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What therapy is useful in the instance of iron overload with transfusions

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Chelation therapy

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Caring for patients with this condition can be challenging because the illness is very unpredictable

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MDS

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Process that is used to remove excess iron acquired from chronic transfusions. The iron is bound to the agent used and excreted in urine. It can also help decrease arterial plaques and remove metals from soft tissues

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Chelation therapy