Pathophysiology - Hematological Disorders

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Hematologic disorders

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Disorders of the blood

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Composition of blood

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50% water, 50% RBC

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Plasma

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Watery suspension
Proteins
Glucose
Byproducts of metabolism

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Plasma Proteins

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Albumin
Globulins
Fibrogen

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Function of plasma proteins

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clotting

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Byproducts of Metabolism

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Urea, creatinine, ammonia

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Serum

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Plasma without the clotting factors

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Normal WBC count

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5-10,000/uL

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Normal Hemoglobin count

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12-16 g/dL

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Normal hemocrit

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35-45%

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Normal platelet count

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150-350,000 mm3

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Things tested for with CBC

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Infections
Malignancies
Bleeding disordersq

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Hemoglobin

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Iron containing, oxygen transport metalloprotein in the red blood cells in the blood

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Hematocrit

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% of packed RBC when a sample is spun

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Platelet

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Cell fragments that contribute to the clotting cascade

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High WBC count finding

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Infection

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Low WBC count finding

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Immunosuppressant drugs or leukemia

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WBC count >45,000

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Inflammed appendix

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Low Hemoglobin levels finding

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Possible internal bleeding

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Hemocrit level - male

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45+%

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Hemocrit level – female

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35-45%

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Low Hematocrit after surgery

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Because pt is pumped full of fluid which alters the RBC concentration

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Erythrocyte

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RBC that is responsible for carrying oxygen and carbon dioxide from the lungs to the tissues

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Leukocytes

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WBC made in the bone marrow
Live about 4 days
5 kinds
granular & agranular

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Granular Leukocytes

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Basophils
Neutrophils
Eosinophils

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Agranular Leukocytes

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Lymphocytes
Monocytes

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Basophils

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Degranulate inside the blood stream releasing histimine during a sensitivity reaction

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Eosinphils

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Attack parasites

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Monocytes

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Ultimate phagocytes

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First line of defense

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Neutrophils

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High neutrophil count indicates…

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Bacterial infections

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Platelet lifespan

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7-10 days

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Where are platelets made

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Liver…thus liver dysfunction results in low platelet count and excessive bleeding

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RBC

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The number of Red Cells as an absolute number per litere

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Mean corpuscular volume

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The average size of the red cells

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Mean corpuscule hemoglobin

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The average amount of hemoglobin per red blood cell

- how bright is the RBC

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What does hemoglobin measurement refer to

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Oxygen carrying capacity

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Low MCV indicates

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Microcytic anemia

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High MCV indicates

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Macrocytic anemia

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Cause of low MCV

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low iron

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Cause of high MCV

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Lack of folic acid, B12, pernicious anemia

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MCH – blue

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Little oxygen

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MCH – red

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High oxygen

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Erythrocytes

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RBC
Look like red dishes, carry oxygen, require iron for oxygen to stick

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Thrombocytes

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Platelets
-Stick to injured blood vessel walls and forms plugs

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Lymphocytes

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Live in the lymph system and either poison or shoot at the enemy

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Stem cells

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Poorly differentiates cells with the potential to become all types of cells

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Blast count

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WBC that utilize resources but do not function

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Anemia

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Either a decrease in number of erythrocytes or in the ability of the erythrocytes to carry oxygen

Can be caused by low number of the cells ot cells that are too small to carry oxygen sufficiently.

They may also be too large to carry oxygen or circulate well

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Types of anemia

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Cytic - size of the cell
Chromic – Hemoglobin concentration

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Normocytic anemia

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Related to some type of bleeding

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Manifestation of anemia

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Reduced oxygen carrying capacity

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Signs and symptoms of anemia

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Tachycardia
Increased stroke volume
Tachypnea
Pallor
Abd pain
Low grade fever
Chest pain
Lethargic
Poor tissue circulation/healing

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Stroke volume

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Amount of fluid with each heart rate

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Cause of abdominal pain with anemia

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Lack of perfusion to the internal organs

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Renin-angiotensin-aldosterone response

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-Decreased oxygen supply
-Renin is secreted from the kidneys
-Renin causes vasoconstriction and release of angiotensin
-Which casues aldosterone secretion from adrenal glands
-Aldesterone causes sodium retention
-Sodium causes water retention
- Water retension causes increased stroke volume

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Pernicious anemia

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B12 deficiency
Caused by inabilityto absorb B12 or the absence of intrinsic factor

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Intrinsic factor

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Enzyme required for the absorption of B12

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Pariental Cells

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Cells in the lining of the stomach which secrete Intrinsic factor

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Pts who are at risk for pernicious anemia

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Gastroectomy, gastritis, vegan diet

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Eval Pernicious anemia

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Schilling test
Assess GI absorption of B12
Drink and measure B12 in urine

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Rx Pernicious anemia

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Monthly injection of B12 (or high oral dosage)

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Pts at risk for folate deficiency anemia (macrocytic-normochromic

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Alcoholics
Malnourished
Lack vegetable intake

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Microcytic anemia

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Iron-deficiency anemia
Low MCV and Low MCHC
-usually caused by slow chronic blood loss, really poor diet, mal absorption in the duedenum or liver disease

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Signs and symptoms of Iron deficiency anemia

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Pallor, fatigue, lethargy, cold intolerance, irritability, menstral regularity, delay healing

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Rx of Iron deficiency anemia

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Iron replacement therapy; blood transfusion

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Normocytic Anemia

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Normal size, but insufficient number

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Aplastic anemia

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Trouble making RBC
Causede by chemo, autoimmune, radiation, benzene

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Posthemorrhagic Anemia

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Pt has lost too many cells as a result of acute blood loss. Needs transfusion and IV fluids to maintain cardiac output

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Hemolytic Anemia

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RBC destruction – possible incopatible transfusion

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Leukocytosis

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- Normal protective response to physiologic stressors
- too many WBC (above 10,000/mm3)

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Leukopenia

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-Count below 5,000/mm3
- Never normal – results from antirejection drugs or leukemia

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Neutropenia

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- Production when production can not keep up with demand due to severe prolonged infection
- classic pt , cancer pt post chemo

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Neutropenic Pts

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-Must always be watched for s/sx of infection

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Neutropenic precautions

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Prophylatic antibiotics
Isolation room
Hand washing
Avoid invasive procedures (including foely, surgery, suppositories
No fresh plants, flowers, fresh fods

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Thrombocytopenia

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Low platelet count

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Causes of Thrombocytopenia

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- Cancer treatment
- autoimmune reaction
- overactive spleen
- problems with bone marrow production

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Thrombocyticpenia s/sx

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Petichiae, echymosis

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Petichiae

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Subcutaneous bleed

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Echymosis

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Bruising

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Thrombocythemia

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Platelet count greater than 400,000/mm3

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Thrombocythemia causes

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Spleen removal

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Which organ regulates platelet production

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Spleen

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How does the pt look who has anemia (clinical manifestations)?

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Heart will beat faster (tachycardia), stroke volume might increase bcse fluid retention because of aldosterone excreted, breathe faster ( tachypnea), Pallor skin bces of low o2 retention, abdominal pain bcse of low O2 supply, low grade fever, chest [pain bcse muscle starved of O2

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What is hemolytic anemia

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Abnormal breakdown of red blood cells, can be caused by incompatable blood transfusion

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Leukocytosis

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too many white blood cells, count above 10,000/mm3. Normal response to infection.

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Leucopenia

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(penia=low) is low count below 5,000/mm32, never normal. Due to anti rejection drugs.

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Leukemia =

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blasts are crowding out the normal ones.

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What are the consequences of neutropenia

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Low number of neutrophils. Production cannot keep up with demand due to prolonged infection. Eg. Cancer/Chemo.#1 problem is worried about infection. Watch out for temperature above 38C , chk blood cultures, chest xray for infection and keep an eye on wounds. Keep PT in own room. Wash hands and sanitize as you enter the room. Avoid invasive procedures. Poor candidates for catherters, suppositories, Shd not have flowers or plants in room, no fresh foods. Avoid surgery.

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Thrombocytopenia

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low platelet count, below 50K, platelets get low when plts are destroyed by cancer treatments. Other causes are auto immune rxn against platelets, and overactive spleen that eats platlets or problems with bone marrow production, or pts with kidney disease have inhospitable circumstances for platlets and it shortens their lives. Pts w platlets below 15K are at risk for spontaneous bleeding and shd not have needle pricks or a regular razor to shave.

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Thrombocythemia

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High platelet count above 400,000/mm3. Caused by absence of spleen because spleen controls number of platelets. Or high platelet count due to dehydration

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What are the clinical manifestations of thrombocytopenia

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Exymosis = bruising or puetekia= broken cappilaries