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Pathophysiology Exam 4-Hematopoietic & Lymphoid Disorders

Pathophysiology Exam 4-Hematopoietic & Lymphoid Disorders

Pathophysiology Exam 4-Hematopoietic & Lymphoid Disorders

Last updated 28 April 2021

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Passing Grades Flashcards

WBC Lifespan
-Varies per cell type.
-Neutrophils (60-80% of WBC count) half life of 4-10 hrs in tissue and 6 hrs in blood once released from the bone marrow
 
RBC Lifespan
-80-120 days once released from the bone marrow
 
 
Platelet Lifespan
-4-5 days once released from the bone marrow
 
Myeloid
-Originate from ___________ stem cells
→Neutrophils
→Monocytes
→RBCs
→Megakaryocytes (Platelets)
 
Lymphoid
-Originate from ________ stem cells
→T-cells
→B-cells
→Natural Killer cells (NK)
 
Myeloid Neoplasm
-Originate in bone marrow stem cells and are released into the blood stream
-Stimulate the overproduction of one or more cell types
-Produced cells may be normal or abnormal in appearance or function
-Overproduction results in a bunch of cells that don't function like they should
 
Chronic Myeloid Leukemias (CML)
-Overproduction of granulocytes (abnormal, don't function like they should, die off) carrying the Philadelphia chromosome (how it's diagnosed) leading to reduced apoptotic cell death
-Onset usually 40-50 years
-Chemotherapy may produce a short remission
-Converts to AML
-Stem cell transplant → LA 50% (survival)
-Survival about 2 years after Dx
 
CML S&S
-Insidious onset signs & symptoms (S&S)
→↑Granulocyte count
→Fatigue (too many WBCs, not enough RBCs-anemia)
→Weight loss (metabolism inc., all nutrients used to treat cancer cells)
→Sweats (from hypermetabolic rate)
→Bleeding (not a lot of platelets)
→Spleenomegaly with abdominal pain. (spleen is overworked)

-Typically no pain associated with chronic blood disorders, pain in acute
 
Acute Myeloid Leukemias (AML)
-Group of malignancies of varying myeloid stem cells
-Presentation of affected cells vary in differentiation and maturation with ↑blasts (baby version of cells)
-80% of Adult Leukemias
-Onset usually 6th decade of life
-LA (left alive) 40-50% for children and 20-30% adults
-Rx - chemotherapy & new monoclonal antibody modalities
 
AML S&S
-Signs & Symptoms have abrupt onset
→Bone pain (b/c of quick onset, bone marrow works hard in short time)
→Anemia (dec. RBCs)
→Thrombocytopenia (lacking platelets, inc. bleeding and bruising)
→Frequent infections esp skin, GI, GU and respiratory tract (making a bunch of abnormal WBCs that can't fight infection; infection more prominent in these places b/c more exposure to outside of body)
 
Lymphoid Neoplasms
-Malignant transformation of lymphoid stem cells
→T-cells
→B-cells
→NK
-Lymph tissue - lymphoma
-Blood - leukemia
 
CML Important Facts
-Philadelphia chromosome
-No pain
-40-50 year onset
 
AML Important Fact
-There is pain
-CML can convert to AML
 
Chronic Lymphoid Leukemias (CLL)
-Usually due to a malignant b-cell precursor (abnormal B-cells; problem with antibodies) that invades lymphoid tissue and bone marrow→prolonged apoptosis
-Indolent onset in 6th to 7th decade
-Usually Dx by accident on routine lab draws
-Rx- with chemotherapy
-Survival ranges from 8-25 years - often die with instead of the dz ("die with it instead of from it")
 
CLL S&S
-Bone marrow
→Anemia (dec. RBCs)
→Thrombocytopenia (dec. platelets-petechiae, bleeding, bruising)

-Lymphoid tissue
→Enlarged painless lymph nodes and/or spleen (swollen, non-tender,unilateral; enlarged, non-tender lymph node in groin)

-Anorexia (b/c of anemia, stomach not getting oxygen)
-Weight loss
-Fatigue
-Frequent infections
 
Acute Lymphoblastic Leukemias/Lymphomas (ALL)
-Majority are malignant transformation of b-cells
-Presents as lymphoblastic leukemia
-Lymphoblasts crowd the bone marrow and suppress the formation of other blood cells
 
ALL S&S
-Abrupt onset
→Bone pain
→Bruising
→Fever (hyper metabolic rate)
→Anorexia
→Fatigue
→Abdominal pain (stomach is oxygen-deprived)
→Activity avoidance (bones hurt)

-Common in children
-LA 85% in kids and 30-50% in adults
-Rx - chemotherapy, bone marrow transplant, monoclonal antibodies
 
Plasma Cell Myeloma (Multiple Myeloma)
-Malignancy of b-cells→↑antibody fragments called Bence Jones proteins
-Multiple tumor sites - bone, liver, spleen, kidneys, lymph nodes
-Satellite tumors in organs, bones
-Onset starts at 40 years
-Higher incidence in males
-Rx - chemotherapy, bone marrow transplant, stem cell transplant
 
Plasma Cell Myeloma (Multiple Myeloma) S&S
-Most S&S (insidious onset) are associated with deposition of the antibody fragments in the bone & kidneys
→High Ca++ in blood or urine
→Renal failure
→Honeycomb bone
→Bone pain
→Fractures
→Anemia
→Bleeding tendencies
→Frequent infections
 
Hodgkin Diseases
-Lymphoma (disease of lymph nodes)
-Malignancies of lymph nodes that produce Reed Sternberg cells from b-cells in association with Epstein-Barr virus
→Supra-diaphramatic - medistinal, cervical, supraclaviculur, axillary
-Exist in four forms
-Predictable metastatic pattern
-Overproduction of abnormal antibodies and underproduction of other blood cells
-Most common in males
-Onset between 20-40 years
-Rx - Chemotherapy and radiation
-90% LA at 10 years
 
Hodgkin Diseases S&S
-Painless lymphadenopathy (swollen lymph nodes)
-Night sweats
-Pruritus (crazy itching b/c so many excess cells can't be cleared, stuff gets released and settles in skin)
-Weight loss
-Anorexia
 
Non-Hodgkin Diseases
-Malignancies arising in lymph nodes that originate in any (b-cells, t-cells or NK) lymph tissue and do not have the Reed-Sternberg cells
-Spread early and in unpredictable paths
-Thought to be associated with some viruses (Burkitts's lymphoma - Epstien Barr-mono)
-Worse than Hodgkins
-LA 50% at 5 years
-If detected in stage I or II good prognosis
-Stage III and IV , most common presentation, poorest prognosis
-Rx - chemotherapy, radiation, bone marrow transplant, monoclonal antibodies.
 
 
Non-Hodkin Diseases S&S
-Present with advanced dz
→Lymphadenopathy (painless)
→Fever
→Night sweats
→Weight loss
→Pruritus
→Infections
→Joint effusions (collection of fluid in joint spaces)
 
Mononucleosis ("mono")
-Epstein Barr Virus (EBV) infection
-Most individuals have been exposed to EBV and have developed some immunity
-Exposure to contact with saliva
 
Mono S&S
-4-6 weeks incubation period
-Pharyngitis
-Lymphadenopathy
-Fever
-Spleenomegaly
-Heptamegaly
-Can't do anything with contact
-Dormant or latent for life
-S&S last from 1- 4 months generally
 
Neutropenia
-Low (<500cells/µL) neutrophils
-Results in increased infections
-Infections can be life threatening
-Requires protection of individuals - neutropenic precautions

-Isolation
-PPE always worn
-No kids or sick visitors
-Disposable plates, etc.
-No plants in room
-Dedicated equipment
-Nothing raw
 
Polycythemias
-Excessive RBC, WBC and platelet production
-Increased blood viscosity
-Hypertension
-Thrombosis
-Mucosal hemorrhage
 
Polycythemia Types
-Polycythemia Vera - malignant
-Secondary Polycythemia - chronic hypoxemia (COPD patients, cigarette smokers, living at high altitudes)
-Relative Polycythemia - dehydration (based on their fluid status)
 
Polycythemia S&S
-HA (less serum-liquid pt. of blood-so thicker blood and can't give off O₂)
-Back pain (less serum-liquid pt. of blood-so thicker blood and can't give off O₂)
-Weakness (less serum-liquid pt. of blood-so thicker blood and can't give off O₂)
-Fatigue on exertion (less serum-liquid pt. of blood-so thicker blood and can't give off O₂)
-Pruritus (excessive cell death, settle in skin)
-Dizziness (dec. oxygen to brain)
-Sweating
-Visual disturbances (vessels in eyes too thick)
-Weight loss
-Parethesias (tingling-not enough oxygen)
-Dyspnea
-Joint discomforts
-Epigastric pain (cells sludging up process)
 
Polycythemia Rx
-Contingent on cause
-Remove the cause
-Phlebotomy
-Chemotherapy
-Myelosuppressive therapy
-Draw off blood and throw away (remove cause)
-Stop smoking
-Control COPD
-Move out of mountains
 
Bleeding Disorders
-Thrombocytopenia
-ITP (Idiopathic thrombocytopenia)
-TTP (thromobocytic thrombocytopenia)
 
Thrombocytopenia
-Deficient Platelets due to:
→Decreased production
→Decreased survival time
→Spleenic Sequestration
→Platelet Dilution
 
 
Thrombocytopenia Etiology
-Folate/B12 ↓
-Radiation
-Chemotherapy
-Drugs
-Bone cancer
-Artificial heart valves
-Spleenomegaly
-Hypothermia
-Massive blood transfusions
-Infections
-DIC
-Lifespan is 10 days for normal platelets
 
Thrombocytopenia S&S
-Petechiae
-Purpura (bruising)
-Intracrainal hemorrhage (stroke)
-Prolonged bleeding time
 
Thrombocytopenia Rx
Remove cause and transfuse (give platelets)
 
ITP
Thrombocytopenia of relatively unknown origin
 
TTP
Thrombosis in small veins lead to deficient platelets
 
Thrombocytosis
Excess release of preformed platelets (transient) (too many platelets)
 
Thrombocytosis S&S
-Paradoxical hemorrhage (platelets being made are not effective)
-Peripheral ischemia
-Pulmonary emboli
 
Coagulation Disorders
-Result from:
→Inappropriate activation of the clotting cascade
→Inappropriate formation or stabilization of the fibrin clot
 
Hemophilia A&B
-Inherited X-linked recessive bleeding disorder (A&B)
-Due to Factor VIII deficiency (A)
-Due to Factor IX deficiency (B)
-Inability to form a fibrin clot
-Hemophilia A is most common
-Deficient in clotting factors
 
Hemophilia A&B S&S
-S&S related to excessive bleeding
→Prolonged bleeding from trauma
→Spontaneous bleeding
→Bruising
→Hematomas (big collection of blood, raised bruise)
→Hemarthrosis (bleeding into joints)
→Hematuria (bleeding into urine)
→GI bleeding (in gut)
→Intracranial bleeding (in head)
 
 
Hemophilia A&B Diagnosis
-based on history, bleeding times and factor assay
-usually diagnosed as kids
 
Hemophilia Rx
-Lifestyle changes
-Replacement therapy
-Factor replacement
 
Von Willebrand Disease
-Similar to hemophilia
-Precursor to Factor VIII
-Autosomal dominant lack of a carrier protein for Factor VIII
 
Von Willebrand Disease S&S
-Epitaxis (nosebleed)
-Rarely hemarthrosis
-Menorrhagia (heavy bleeding during menstrual cycle)
-GI bleeding
 
Von Willebrand Disease Diagnosis
Hx, bleeding times and factor assay
 
Von Willebrand Disease Rx
Desmopressin, hormonal suppression, replacement therapy
 
Vitamin K Deficiency
-Vitamin K is necessary for several clotting factors
-Infants are deficient due to a sterile gut (bacteria produce vitamin k), liver immaturity (processes vitamin k) and low dietary intake of vitamin K
-Need functioning liver to make vitamin K
 
Vitamin K Deficiency S&S
-Melena (blood in stool)
-Hematuria
-Intracranial hemorrhage
-GI bleeding
-Menorrhagia
 
Vitamin K Deficiency Rx
Vitamin K replacement
 
Disseminated Intravascular Coagulation (DIC)
-Like thrombocytopenia but more fibrin
-Widespread intravascular thrombosis →widespread hemorrhage due to consumption of coagulation factors
-Occurs 2ndary to malignancies, sepsis, snake bite, abruptio placenta (placenta tears from uterine wall), trauma, crush injuries, burns, shock, severe liver disease, incompatible blood transfusion
-Happens b/c of big stressor (infection, trauma, etc.)
 
 
DIC S&S
-Petechiae
-Ecchymoses
-Orifice bleeding
-Needle stick site bleeding
-Dyspnea
-Hemoptysis
-Renal
-*Crazy bleeding
 
DIC Diagnosis
Clinical presentation and a series of coagulation studies; history of some stressor
 
DIC Rx
-Remove/correct cause
-Replacement of clotting factors
-Drug therapies
 
Anemia
-Disease indicator
-Low hemoglobin
-Low RBC
→Diminished O2 carrying capacity
→Tissue hypoxia thus fatigue, weakness, angina, pallor
→Hypotension, lightheadedness, tinnitus (ringing in ears

-Compensatory mechanisms (organs sense there isn't enough oxygen)
→↑HR, ↑RR, ventricular hypertrophy
→Bone pain

-Pale, weak, tired
 
Classification of Anemias: -cytic
-morphology-size, shape of cell
-normo-normal
-macro-big cell
-micro-small cell
 
Classification of Anemias: -chromic
-color-iron determines color
-normo
-hypo-not a lot of color
-hyper-a lot of color
 
Types of Anemia
-Hemolytic
-Blood loss
-Hemoglobinopathies
 
Blood Loss Anemia
-Acute
→Normocytic
→Normochromic
→Dilutional

-Chronic
→Iron-deficiency from depletion of iron-usually from GI tract
→Microcytic
→Hypochromic
 
Hemolytic Anemias
-Premature destruction of RBC (crush injury, incompatible transfusion)
→Primarily in the spleen
→Also in the vascular space

-Retention of the by products of RBC lysis
-Increase erythropoieses
-Inherited or acquired
-Results in spleenomegaly, jaundice, and bilirubin gallstones
 
Inherited Hemolytic Anemia
-Sickle Cell Anemia
-Thalassemia
 
Sickle Cell Anemia Etiology/Pathogenesis
-Autosomal Recessive Disorder
-Abnormal Hg - HgS
-Common in African Americans
-RBCs sickle under stress/triggers
→Low oxygen
→Low vascular volume
→Cold stress
→Infections
→Acidosis
→Extreme physical exertion

-B/c of shape, they all get stuck on each other and plug up blood vessels and shut down tissues

-Vaso-occlusion from cells that clump - severe pain.

-Organ dysfunction
→Extremities - necrosis
→Joints - infarcts
→Lungs - acute chest syndrome
→Spleen - ↑infections
 
Sickle Cell Anemia S&S
-Hemolysis of sickled cells
-Spleenomegaly
-Asplenia (spleen not functioning)
-Hyperbilirubinemia
-Sludging of RBCs
-Necrosis from occlusions/infarcts
-Infections from necrosis
-Infection leading cause of morbidity and mortality especially before three years of age
 
Sickle Cell Rx
-avoid triggers
-Hydroxyurea - stimulate HgF (fetal Hgb-doesn't sickle) production
-Stay hydrated
-Don't smoke
-Stay away from sick people
 
Thalassemia Etiology/Pathogenesis
-Individuals of *Mediterranean, Asian, or African American descent
-Deficient Hg production & hypochromic microcytic anemia
-Heinz bodies in the bone marrow impair RBC production
-Accompanying hypercoaguability leads to strokes and pulmonary emboli
-Growth retardation without blood transfusions begun early
-↑hematopoiesis → bone marrow expansion → bony abnormalities (chipmonk faces, fractures)
-Spleenomegaly
-Hepatomegaly
-Iron toxicities
 
Acquired Hemolytic Anemia
-Lysis of RBCs due to exogenous factors
→Blood transfusions
→Drugs
→Chemicals
→Venoms
→Certain Infections
→Prosthetic heart valves
→Burn injuries
→Vaculitis
 
Anemias of Deficient RBC Production
-Result from decreased RBC production
→Lacking adequate nutrients
→Failed bone marrow
 
Types of Anemias of Deficient RBC Production
-Iron deficiency anemia
-Megaloblastic Anemia
-Vitamin B12 deficiency
-Folic Acid Deficiency
-Aplastic Anemia
-Chronic Disease Anemias
 
Iron Deficiency Anemia
-Iron is recycled when RBCs are brokendown
-Chronic blood loss most common cause
-Occurs also during growth spurts and pregnancy
 
Iron Deficiency Anemia S&S
-Low H & H (Hg and hemaocrit)
-Low iron stores
-Low RBC count
-Microcytic hypochromic RBCs
-Fatigue
-Weakness
-Palpitations (early heart beat b/c heart is deprived of oxygen and irritated)
-Dyspnea
-Angina
-Brittle hair and nails
-Increased HR
-Waxy pallor
 
Megaloblastic Anemias Etiology
-Alcoholics
-Pregnant women
-Anorexic
 
Megaloblastic Anemias Pathogenesis
-Large RBCs owing to Folic Acid deficiency or Vitamin B12 deficiency
-Folic Acid and Vitamin B12 are necessary for DNA synthesis
-Results in weak RBCs
-Vitamin B12 deficiency anemia is accompanied by neuronal changes that lead to dementia and other neurological impairment
 
Aplastic Anemia Etiology
-Bone marrow depression leads to low RBC, WBCs & platelets
-Causes:
→Radiation
→Drugs
→Chemicals
→Toxins
→Infection
→Autoimmune reactions

-Bone marrow not growing any cells
 
Aplastic Anemia Clinical Manifestations
-Sudden or insidious onset
→Weakness
→Fatigue
→Pallor
→Petechiae
→Eccochymoses
→Bleeding
→Frequent infections
 
Aplastic Anemia Rx
-Bone marrow transplant
→Graft vs Host reactions
-RBC transfusions
-Antibiotics
-Immunosuppresion
 
Chronic Disease Anemia
-Renal failure
-Cancer
-Chronic infection
-AIDS
-Rheumatoid arthritis
-Systemic Lupus erythematosus
-Hodgkins Dz

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