Module 10: Alterations Of RBC’s, Platelets, WBC’s And Lymphoid/Hemostatic Function (b) Flashcards
Anemia
-Definition
- Reduction in total number of erythrocytes in the circulating blood OR in the quality or quantity of hemoglobin
- Impaired Erythrocytes production
- Acute or chronic blood loss
- Increased erythrocytes destruction
- Combination of all the above
Anemia
-Classifications
- Etiology
- Morphology — Mainly based on MCV -macrocytic, microcytic, normocytic
- Hemoglobin content —Identified by ending in “chromic”
Other Descriptors
- Anisocytosis — RBCs are present in various sizes (RDW) — Accelerated RBC destruction
- Poikilocytosis — RBCs are present in various shapes (Peripheral blood smear) — Possible Blood borne cancers — Ex: SICKLE CELL
Anemia
-Manifestations
- MAJOR physiologic manifestation is HYPOXIA TEST
2. Classic Sx’s of anemia — Fatigue, weakness, dyspnea, and pallor
Anemia
-Compensatory Mechanisms
- CV Function — Increased preload, HR, & Stroke volume; reduced Afterload —Vasodilation is hypoxia induced — Lower blood viscosity
- Respiratory Function — Dyspnea
- Nervous System Function — myelin degeneration & Parasthesias
- GI Function — Pain, N/V anorexia
Normochromic-Macrocytic Anemias **
-Info
- Megaloblastic anemias — RBCs are unusually large
- Defective DNA synthesis — D/t deficiencies in vitamin B12 or folate
- DNA synthesis is altered while RNA processes occur at normal rate — Results in unequal growth of nucleus and cytoplasm
Normochromic-Macrocytic Anemias **
-Vitamin B12 Deficiency Anemia
- Pernicious anemia is MOST COMMON subtype — Results from lack of INTRINSIC factor from gastric parietal cells
-Main Cause is: Atrophic Gastritis
—Autoimmune forms — Parietal cell antibodies — Intrinsic factor antibodies
-H. Pylori infection destroys parietal cells
-Prolonged PPI and H2 blocker use — Gastric acid is needed to cleave Vit B12
-Gastrectomy — Loss of gastric parietal cells
Normochromic-Macrocytic Anemias **
-Pernicious Anemia (Vit B12) Clinical Manifestations
- Weakness/Fatigue
- NEUROLOGIC Sx’s from nerve demyelination — NOT reversible even w/ Tx
- Paresthesias of feet and fingers — difficulty walking
- Loss of appetite, abdominal pain and weight loss
- Sore tongue that is smooth and BEEFY RED — Secondary to atrophic glossitis
- Often unrecognized in older adults d/t subtle slow onset and presentation (Develops over 20-30 yrs)
Normochromic-Macrocytic Anemias **
-Folate Deficiency Anemia
- Folate is essential vitamin for RNA and DNA synthesis
- Absorption of folate occurs in UPPER SMALL INTESTINE; self dependent
- Common in alcoholics and individuals w/ chronic malnourishment —Folate is stored in the liver
- Associated with FETAL NEURAL TUBE DEFECTS
Hypochromic-Microcytic Anemias
-Definition/Info
- Characterized by red cells that are abnormally small and contain reduced amounts of hemoglobin
R/T
-Disorders of iron metabolism
-Disorders of porphyrin and heme synthesis
-Disorders of globin synthesis
Hypochromic-Microcytic Anemias
-Iron-Deficiency Anemia
- Most common type of anemia worldwide
- Highest risk — Older adults, women, infants — Black females living in urban poverty HIGHEST RISK
- Associated w/ cognitive impairment in children
- Iron deficiency anemia and folate deficiency anemia are two LEADING causes of anemia in pregnancy
Hypochromic-Microcytic Anemias
-Iron-Deficiency Anemia Causes
- Inadequate dietary intake
- Chronic blood loss — Most Common in developed countries
- Chronic parasitic infections — Developing countries
- Metabolic or functional iron deficiency
- Menorrhagia — Excessive bleeding during menstruation
Ask Pt’s :: Any blood in stool?
Hypochromic-Microcytic Anemias
-Iron-Deficiency Anemia Clinical Manifestations
- Fatigue, weakness, SOB
- Pale earlobes, palms, and conjunctivae
- Brittle, thin, coarsly ridged and spoon-shaped nails — Concave or KOILONYCHIA **
- Red, sore, painful tongue — Severity of tongue pain coincides w/ severity of anemia
- Angular stomatitis — Dryness and soreness in corners of the mouth
- Symptomatic when Hgb is 7-8g/dl
Hypochromic-Microcytic Anemias
-Sideroblastic Anemia
- Group of disorders caused by defect in mitochondrial heme synthesis
—Altered mitochondrial metabolism causes ineffective iron UPTAKE and results in dysfunctional hemoglobin synthesis - Ringed sideroblasts in bone marrow are diagnostic
- Clinical manifestations — Hemochromatosis & Splenomegaly & Hepatomegaly
Normochromic-Normocytic Anemias
-Definition/Info
- Characterized by RBCs that are relatively normal size and hemoglobin content, but INSUFFICIENT NUMBER
- Least frequent w/ no common cause
Normochromic-Normocytic Anemias
-Aplastic Anemia
- Pancytopenia — Reduction or absence of ALL 3 types of blood cells (Ex: WBC, RBC, and PLT)
- Most aplastic anemias are AUTOIMMUNE disorders — Some d/t chemical exposure to benzene, arsenic, chemotherapy drugs
- Pure RBC aplasia — Only RBCs are affected
Normochromic-Normocytic Anemias
-Aplastic Anemia Patho
- AUTOIMMUNE destruction of hematopoietic stem cells or their progenitors at various stages of differentiation by CYTOTOXIC T-CELLS
- Clinical Findings — Characteristic lesion is a hypo cellular bone marrow that has been REPLACED WITH FAT
- Clinical Manifestations
- Hypoxemia, pallor — occasionally brownish pigmentation of the skin
- Weakness w/ FEVER and dyspnea w/ rapidly developing signs of HEMORRHAGING if PLT’s are affected
Normochromic-Normocytic Anemias
-Post-Hemorrhagic Anemia
- Acute blood loss from vascular space
2. Manifestations depend on severity of blood loss — If blood loss exceeds 2 liters, Shock sets in
Normochromic-Normocytic Anemias
-Hemolytic Anemia
- Accelerated destruction of RBCs — Congenital vs acquired
- Autoimmune hemolytic anemias
—Autoantibodies against antigens normally on surface of erythrocytes
Normochromic-Normocytic Anemias
-Autoimmune Reactive Antibody Types
- Warm-Reactive Antibody Type — IgG which binds at normal body temp 40 degrees C
- Cold agglutinin Type — IgM — Optimally binds at 31 degrees C — Can occur after Infections — Ex: Mono, mycoplasma PNA, TB
—Occurs in lower temp areas — Ex: Fingers, toes, and ears — Can lead to gangrene - Cold Hemolysin Type — IgG Occurs in children <10 yrs old — Intravascular hemolysis s/p exposure to cold — IgG auto-antibodies
—Linked with Mums, measles, varicella, mycoplasma PNA
Normochromic-Normocytic Anemias
-Drug-Induced Hemolytic Anemia
- Result from allergic reaction against foreign antigens
—Ex Meds: Penicillin, Cephalosporins (90%), hydrocortisone
Normochromic-Normocytic Anemias
-Anemia of Chronic Disease (Inflammation)
- Seen in — AIDS, RA, SLE, Hepatitis, renal failure, & Malignancies
- Pathological Mechanisms TEST
- Decreased erythrocytes life span
- Suppressed production of erythropoietin
- Ineffective bone marrow response to erythropoietin
- Altered iron metabolism - During inflammation, Neutrophils release LACTOFERRIN to bind to iron to reduce it’s availability for bacteria
Myeloproliferative RBC Disorders
-Polycythemia
- Relative Polycythemia — Dehydration — Fluid loss results in relative increase of RBCounts & Hgb and Hct values — Resolves w/ fluid intake
- Polycythemia Vera
- Chronic neoplastic, NONMALIGNANT condition
- Splenomegaly
- Donate blood is Tx
Myeloproliferative RBC Disorders
-Iron Overload
- Primary — Hereditary hemochromatosis
-Common inherited, autosomal recessive disorder of iron metabolism
-Excess iron deposited in liver, pancreas, heart, joints, endocrine glands, causing TISSUE DAMAGE
LEADS TO — Bronze skin, Hepatomegaly, DM, Cardiomegaly
-TX is phlebotomy, DO NOT take vitamin C or Iron
OR
2. Secondary — Cytoblastic anemia, Aplastic anemia, Dietary overload
Neutropenia
-Definition/Info
Reduction in circulating neutrophils
- Primary — Kostmann & box syndrome
- Congenital — Cyclic neutropenia and neutropenia w/ congenital immunodeficiency diseases — Multiple syndromes
- Acquired — Hypoplastic anemia, aplastic anemia, leukemia’s, lymphomas, myelodysplastic syndrome - Secondary — Lower count from other disorders —Ex: Immune disorders and drugs — Lupus, Splenomegaly, CA meds
Lymphocytosis & Lymphocytopenia
- Lymphocytosis
- Acute viral infections — EBV
- Other causes — Leukemia, lymphomas, some chronic infections - Lymphocytopenia
—Causes — Immune deficiencies, lymphocyte destruction from drugs, viruses, or radiation — AIDS
Infectious Mononucleosis
-Info
- Acute, Self-limiting infection of B-Lymphocytes transmitted by saliva through personal contact**
- Commonly caused by the Epstein-Barr Virus EBV (85%) — B lymphocytes have EBV receptor site
- Other viruses that cause IM
- Cytomegalovirus CMV — Think CMV for IM infection in person >40 yrs old
- Hepatitis
- Influenza
- HIV
Infectious Mononucleosis
-Classic Triad
- Fever, Tonsillar enlargement, & Lymphadenopathy of cervical or auricular lymph nodes (Found 98% of the time together in positive IM)**
- Diagnostic is the MONOSPOT qualitative test for heterophilic antibodies 98% specific
Leukemia’s
-Info
- Malignant disorders of the blood and blood-forming organs
- Uncontrolled proliferation of malignant leukocytes
- Overcrowding of bone marrow
- Decreased production and function of normal hematopoietic cells - Classification
- Predominant cell of origin — Myeloid or lymphoid
- Rate of progression — Acute or chronic
Leukemia’s
-Acute Vs Chronic
- Acute Leukemia
—Presence of undifferentiated or immature cells — Usually BLAST CELLS
—RAPID onset w/ narrow window in which to treat - Chronic Leukemia
—Predominant cell is MATURE but does not function normally
—SLOW progression
SEE PHOTO IN PHONE**
Leukemia’s
-Acute Lymphocytic Leukemia (ALL)
- MOST COMMON childhood leukemia
- Greater than 30% lymphoblasts in the bone marrow or blood
- Genetic anomaly — PHILADELPHIA CHROMOSOME
- Reciprocal translocation results in abnormal chromosome — Between chromosomes 9 and 22
Leukemia’s
-Acute myelogenous Leukemia (AML)
- MOST COMMON adult leukemia (Mean age 67 yrs)
Causes
- Abnormal proliferation of myeloid precursor cells
- Decreased rate of apoptosis
- Arrest in cellular differentiation
RISK factors
- Down syndrome
- Exposure to radiation, benzene, and chemotherapy
- Hereditary conditions
Leukemia’s
-Clinical Manifestations of ACUTE leukemia’s
- Fatigue — Anemia
- Bleeding — Reduced PLTs
- Fever — Infection from decreased healthy WBC’s
- Bone pain — Proliferation of lymphoblasts and myeloblasts stretch periosteum of the bone
- CNS — Leukemic infiltration — Cerebral bleeding and HA, blurred vision, meningeal irritation
Leukemia’s
-Chronic Leukemia’s
- Chronic Lymphocytic Leukemia (CLL) — COMMON in adults older than 50 yrs (70% Asymptomatic at diagnosis
—Affects monoclonal B lymphocytes — One single ancestral cell mutated
—FAMILIAL tendency - Chronic Myelogenous Leukemia (CML) — Usually Dx’d in adults (60 -80yrs old)
—Philadelphia chromosome is OFTEN PRESENT and BCR-ABL1 causes initiation of CML
Leukemia’s
-Chronic Lymphocytic Leukemia manifestations
- Asymptomatic at time of Dx
- Lymphadenopathy is MOST COMMON finding
- Increased risk of infection w/ encapsulated bacteria
Leukemia’s
-Chronic Myelogenous Leukemia
Infections, fever, and weight loss
- Chronic Phase — 2-5 years — Sx’s may not be apparent
- Accelerated phase — 6-18 months — SPLENOMEGALY is primary sx
- Terminal Blast Phase — “BLAST CRISIS” — Survival ONLY 3-6 months
Malignant Lymphomas
-Info
- Neoplasm of malignant lymphocytes in the lymphoid system
- Two major categories
—HODGKIN lymphoma — Linked to EBV**
—Non-Hodgkin lymphoma - Result from genetic mutations or a viral infection
Leukemia = CA in the bone marrow and blood Lymphomas = CA is in lymph nodes and other tissues (Thymus & Bone marrow)
SEE PICTURE IN PHONE
Malignant Lymphomas
-Hodgkin Lymphoma
- Proliferation of abnormal B cells (Reed-Sternberg cells) that may be linked to EBV infectionTEST
- Reed-Sternberg cells in the lymph nodes
- Derived from malignant B cells that usually become binucleate
- Necessary for the dx but not specific to Hodgkin lymphoma
- Release cytokines — iL10
Malignant Lymphomas
-Hodgkin Lymphoma Clinical Manifestations
- Enlarged PAINLESS neck lymph nodes
- Lymphadenopathy, causing pressure or obstruction
- Mediastinal mass
- Fever, weight loss, night sweats, pruritus, fatigue
Tests
-CXR, Lymphangiography, BIOPSY (GOLD STANDARD)
Malignant Lymphomas
-Non-Hodgkin Lymphoma
- Generic term for a diverse group of lymphomas — B-cell neoplasms make up 80 - 90%
Malignant Lymphomas
-Non-Hodgkin Lymphoma Clinical Manifestations
- Localized or generalized painless lymphadenopathy
- Retroperitoneal and abdominal masses w/ Sx’s of abdominal fullness and back pain
- Ascites and Leg swelling
- Dx Tx — BIOPSY
Multiple Myeloma
-Definition/Info
- Plasma cells proliferate in the bone marrow
- Increased osteoclastic bone destruction
- Anemia 72%, Bone lesions 80%, Hypercalcemia** MOST CONCERNING, renal failure
Clinical Manifestations
- Cortical and medullary bone loss
- Skeletal pain
- Recurring infections d/t loss of humoral immune response
Tx is mostly supportive — POOR PROGNOSIS
Disorders of Platelets
-Thrombocytopenia
- Platelet count <100,000/mm
- Caused by — Hypersplenism, Autoimmune dz, hypothermia, & viral/bacterial infections that cause DIC
- Clinical Manifestations — Petechiae and Purpura — Frank bleeding from mucous membranes (epistaxis, hematuria, Menorrhagia, bleeding gums)
Disorders of Platelets
-Immune Thrombocytopenic Purpura ITP **
- IgG antibody targets PLT glycoproteins TEST
- One of the MOST COMMON childhood bleeding disorders — s/p viral infections
- Manifestations — Petechiae & Purpura, progressing to major hemorrhage
Disorders of Platelets
-Essential (Primary) Thrombocythemia
- PLT count > 400,000/mm
- Myeloproliferative disorders of PLT precursor cells — Megakaryocytes in bone marrow are produced in EXCESS
- Microvasculature Thrombosis — More concerning