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Hematology: Pathophysiology

Terms in this set (72)

Normal WBC count?
5,000-10,000
Normal neutrophil count?
3,000-7,000 (of the total WBC count)
What cell is the first line of defense against infection?
Neutrophils. There is an immature release of cells in acute infection.
Role of eosinophils?
Bind to parasites, ^ in allergic states, or in patients on steroids.
Role of Basophils?
Least understood. Increased in allergic states. Respond to histamine.
Lymphocytes: role? Describe B and T cells.
- 1,000-1,400 of total cell count
- Humoral and cell mediated immunity
- B cells (antibody producers)
- T cells (direct attacker cells)
When do B cells (referring to type of lymphocytes) increase?
B cells increase in leukemia and in multiple myeloma.
How many platelets are there?
150,000-340,000
How many RBCs are there?
4.2-6.2 million
What are blast cells?
They are immature or "band" cells. Not fully functional.
Shift to the left...
Leukemoid reaction. The term leukemoid reaction describes an increased white blood cell count, or leukocytosis, which is a physiological response to stress or infection (as opposed to a primary blood malignancy, such as leukemia).
Shift to the right...
Back to normal cell distribution.
What is anemia?
Anemia is a lower than normal number of RBCs; OR, it refers to a decease in the quality or quantity of hemoglobin in RBCs.
Describe the most severe s/sx of anemia.
Fainting, chest pain, angina, heart attack.
How are anemias classified?
- Classified according to size (micro-, macro-, norm-) of the RBC and according to color (normochromic or hypochromic)
What are the three types of microcytic/hypochromic anemias?
1. Iron deficiency anemia - lack of iron for cell formation
2. Sideroblastic anemia - dysfunction in iron uptake in RBCs
3. Thalassemia - impaired hemoglobin synthesis
What are the two types of macrocytic/normochromic anemia?
1. B12 anemia (pernicious) - lack of IF leads to lack of B12 absorption
2. Folate deficiency - lack of folic acid leads to premature cell death
What is the one type of microcytic/normochromic anemia?
An erythropoietin deficiency from kidney failure.
Describe pernicious anemia.
Most common megaloblastic (aka macrocytic) anemia. D/t a lack of intrinsic factor > lack of B12 absorption.
What is the age of onset of pernicious anemia? What are the future consequences of lack of B12?
Age of onset is later (age 20-30). Watch for weight loss, "beefy tongue," jaundice, pallor. Later will have right sided heart failure and nerve damage.
Cure for pernicious anemia?
Lifetime supplementation of B12 parentally (will not absorb PO or SL).
Describe folate deficiency.
Folate is needed for RBC DNA/RNA synthesis. Obtained from green leafs. Folate not stored in body. NEed constant supply. Most crucial for prenatal health.
S/sx of folate deficiency?
- Tiredness, HA
- Sore mouth and tongue fissures; ulceration of tongue/lips (stomatitis)
- Diarrhea, dysphagia, dysphagia
Microcytic/hypochromic anemia: iron deficiency
Iron deficiency anemia is the most common form of anemia.
What is the most common form of iron deficiency anemia?
Pregnancy, chronic blood loss d/t gastric/duodenal ulcers, neoplasms.
What is sideroblastic anemia?
Associated with inefficient iron uptake resulting in abnormal hgb synthesis. Irons build up in tissues. Iron overload > enlarged liver and spleen.
Thalassemia: described
- Genetic defect that can be inherited; defect in the production of the chain of adult hemoglobin
S/sx thalassemia...
- Growth failure, bone deformities, enlarged liver, growth retardation, "chipmunk" face due to expansion of bone to accommodate hyperplastic marrow
Normocytic/normochromic: RBCs normal in size and appearance - just too few of them...
Aplastic anemia: reduction of all cell types - red blood cells, white blood cells, and platelets > pancytopenia. ***Only anemia that impacts ALL cell types.
Normocytic/normochromic: post hemorrhagic anemia
- In acute hemorrhage, RBCs are normal in color and size
- D/t lack of blood volume
Anemia of Chronic Inflammation...
- Clients with chronic infections such as AIDS, RA, SLE, malignancies
- Causes are decreased RBC life span
What is polycythemia vera?
- Chronic overproduction of RBCs (frequently WBCs and platelets also)
- Rare disease seen in Eastern European Jewish origin
- Proliferation of neoplastic, nonmalignant proliferation of bone marrow stem cells
Consequences of PV?
- S/sx are related to increased blood volume > hyper-coagulability
- Clots form, obstruct blood vessels results in ischemia and infarction
- Plethora (ruddy face and skin tone)
- ***Classic symptom: intense, painful itching that is intensified by heat or exposure to water
What is leukocytosis?
- Leukocyte count higher than normal.
Leukocytosis: what is a left shift?
An increased number of immature neutrophils/band cells.
Leukopenia: is it normal? What is it?
It is a count of WBC lower than normal. When count falls below 1,000. Leukopenia is NEVER normal.
At what number does the risk for severe infection increase dramatically (with leukocytosis)?
Risk for infection increases dramatically when number falls below 500.
Neutropenia: what is it? What is the number?
Neutrophil count less than 2,000. Starvation, anorexia can cause it.
Infectious Mononucleosis: what is it?
- Acute infection B lymphocytes
- Common in young adults 15-35 years
- 50-85% of low socioeconomic environments
Leukemia: what is it?
A group of bone marrow diseases involving uncontrolled proliferation of undifferentiated (immature) white blood cells (malignant leukocytosis) BLAST CELLS.
Leukemia: what are the different ways to classify leukemia?
1. Myelocytic (bone) and lymphocytic (lymph)
2. Acute (immature cells) or chronic (mature cells, develop over time)
3. Abbreviations: CML, AML; CLL, ALL
Leukemia: s/sx
- Exact cause unknown; tends to reappear in families
- Leukocytosis (^^^ in all WBC)
- Anemia
- Decreased platelets
- Bleeding gums
- Bruising
- Fever
- Lymph nodes/liver/spleen enlargement
- Bone pain
- Elevated uric acid
- Night sweats
- Lymphadenopathy: enlarged lymph nodes; can be widespread or localized
What is the Philadelphia marker used to diagnose?
Used to identify chronic myelocytic leukemia.
What is multiple myeloma?
- B cell cancer
- Originates in bone marrow > moves through lymph and affects entire body
- ^ in black population
- Progressively destroys corticol bones > bone pain and pathological fractures
What does multiple myeloma lead to in the blood?
Hypercalcemia.
Multiple Myeloma patient: What type of proteins should you look for in the urine?
Look for Bence-Jones proteins in the urine. These proteins damage the kidneys.
Predominant s/sx of multiple myeloma?
Hypercalcemia, renal failure (d/t Bence-Jones proteins in urine), anemia and bone lesions/pain, pathological fractures.
What is Hodgkin's lymphoma (cancer of what)?
It is a cancer of the lymph tissues found in the lymph nodes, spleen, liver, and bone marrow.
What is the first sign of Hodgkin's lymphoma?
First sign of this cancer is an enlarged, painless lymph node which appears without known cause.
What is the diagnostic factor in diagnosing Hodgkin's lymphoma?
The presence of Reed-STernberg cells (large, multi-nucleated lymph cells).
How is the severity of Hodgkin's lymphoma calculated?
Using the Cotswold Staging System. This is also how treatment is determined.

Cotswold Staging System:
I Single Lymph Node
II 2 or more nodes same side of diaphragm
III Lymph nodes involved on both sides of diaphragm
IV Disseminated involvement
75% will be cured, survival decreased with high stages
What is Non-Hodgkin's lymphomas? What sites are usually involved?
- B-cell neoplasms
- Victims commonly middle aged
- Sites of involvement: usually peripheral lymph nodes > most commonly cervical, axillary, inguinal, femoral chains
Non-Hodgkin's Lymphoma: what s/sx does the pt. experience if lymphomas occur in the retroperitoneal and abdominal spaces?
Feelings of abdominal fullness, back pain, leg pain and ascites.
What is essential to remember about non-hodgkin's lymphoma?
It is rarely localized.
Burkitt Lymphoma: what are identifying factors?
- Abdominal swelling
- B cell symptoms (night sweats, fever, weight loss)
- Etiology: associated with Epstein Barr virus (90% and greater) and frequently occurs in immunocompromised patients
What is a splenic alteration?
- Enlargement of the spleen may be a sign of an underlying condition
- Results from hematologic alterations resulting from spleen sequestering red cells, white cells, and platelets and a consequent reduction in circulating cells > anemia develops
What constitutes thrombocytopenia? What is severe?
- Platelet count of less than 150,000
- Severe bleeding occurs when <10,000
What is the most common medication resulting in thrombocytopenia?
Heparin-induced thrombocytopenia.
What deecreases the risk of heparin-induced thrombocytopenia?
Use of low molecular weight heparin.
What is the concern with thrombocytopenia?
The thinned blood may pool - developing clots - this can cause a DVP or pulmonary emboli. > CVA or MI
What are other forms of thrombocytopenia?
- Idiopathic thrombocytopenia purpura (ITP) - autoimmune
- TTP: thrombotic platelets aggregate and occlude arteries - seen in HELLP syndrome and OB
- Altered platelet function
What is DIC?
- Triggered by major trauma, shock, sepsis, transfusions, malignancy, pregnancy, hypoxia.
- Endothelial damage, tissue damage, and direct activation of factor X > leading to micro-clotting in body
- Micro-clotting: leads to occlusion of vessels leading to tissue hypoxia/death of organs
- Body tries to compensate - activates coagulation cascade > clotting breakdown mechanisms released > bleeding in sclerae, development of pethechiae throughout body
S/sx of DIC?
- Oozing from puncture sites, hemorrhage, petechiae
- Altered LOC, coma
- Massive bleeding in GI, weakness
- PUlmonary embolism, cyanosis, hypoxemia
- Hematuria, oliguria, renal failure
What is the Virchow triad?
This analyzes three factors as risks for spontaneous thrombi:
1. Injury to blood vessel endothelium
2. Abnormalities in blood flow
3. Hyper-coagulability of blood
What is sickle cell anemia?
- Abnormal hemoglobin "S" shape
- These abnormal hemoglobin are non-functional - can not be oxygenated
- This deoxygenation and dehydration rigger sickling > clumping
- Sickling triggered by infection, OSA, dehydration, acidosis
What are the four types of sickle cell crisis?
1. Vasoocculsive or thrombotic crisis - most common type; begins with sickling (thickening/clumping of RBC); jam in circulation leading to thrombosis and infarction
2. Sequestration crisis: blood pools in spleen/liver - 50% mortality
3. Aplastic crisis: profound anemia, RBC live 10-20 days; bone marrow nonfunctional
4. Hyper-hemolytic - rare
What is the most important thing to remember about sickle cell anemia?
Genetic counseling is important!
Vasooclusive crisis: what is most common s/sx?
Extreme pain d/t tissue lack of oxygen and death.
What is hemophilia A d/t?
D/t lack of factor 8.
What is hemophilia B called?
Christmas disease. D/t lack of factor 9.
Childhood Leukemia?
- 85% of kills have ALL (acute lymphocitic leukemia)
- Peak is 2-6 years; more common in white
Describe ALL (acute lymphocytic leukemia) in children?
Decreased RBC/platelets/WBCs; pallor; fatigue; petechiae; purpura; bleeding gums; fever; WBC greater than 200,000 can lead to leukostasis and intravascular clumping and can infarct lung/brain
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