Hematology & Anemias
Blood is a connective tissue
The blood is: 55% plasma, 45% formed elements (44% RBCs)
H & H: Hemoglobin and hematocrit, used for anemia testing
Hematocrit: % of any RBCs in any volume of blood (max is 44%)
CBC: Complete blood test; measures hemoglobin, hematocrit, platelet and WBC count, used for infection
ALWAYS ordered with a differential
“Inability of the blood to supply the tissues with adequate oxygen for proper metabolic function”
Either inadequate hemoglobin or RBC can lead to anemia
Hg: hemoglobin concentration
Hct: hematocrit concentration (packed cell volume)
RBC: red blood cell count
ANEMIA IS NOT A DISEASE; expression of an UNDERLYING disorder/disease
→ Normal Hg and Hct Levels
Hg < 13 in men; Hct <41%
Hg < 12 in women; Hct <36%
Hg <14 in men; Hg <12 in women (with malignancy)
Men | Women | |
---|---|---|
Hemoglobin (Hb) | 13.2-16.7 | 11.9-15.0 |
Hematocrit (Hct) | 38-48% | 35-44% |
Red Cell Count | 4.2-5.6 | 3.8-5.0 |
Reticulocyte Count | 0.5-1.5 | 0.5-1.5 |
Mean Cell Volume (MCV) | 81-97 | 81-97 |
Lastly, the anemia morphology:
Size: Macrocytic, microcytic, normocytic
Macro= the entire RBC
Micro= hemoglobin only
Hemoglobin content: Normochromic and hypochromic
Microcytic, MCV < 80 (reduced iron availability)
Normocytic, 80 < MCV < 100
Macrocytic, MCV > 100 (liver disease/B12 deficiency)
Hematocrit: volume of packed RBCs per unit of blood, expressed as a % (ex: 44% is the max)
38-44% is in normal range
Reticulocyte: young RBC, not functionally mature, anemia due to hemolysis or bleeding is characterized by reticulocytosis
Normal value: ~1%
Increase in reticulocytes is due to compensation to bring up the RBC
Mean corpuscular volume (MCV): reflect average size or volume of RBC, will tell if the patient is micro, macro, or normcytic
Mean cell hemoglobin concentration (MCHC): indicates the concentration of Hb in the average RBC, or the ratio of the weight of the Hb to the volume in which it is contained (chromicity/color)
Easy fatigue and loss of energy
Tachycardia, rapid heart rate
Shortness of breath
Headache
Difficulty concentrating
Dizziness
Pale skin
Leg cramps
Insomnia
Characterized by unusually large stem cells
Can also be termed megaloblastic anemias
→ PATHOGENESIS:
Defective DNA synthesis in rapidly dividing cells
Caused by B12 or folate deficiencies
Vitamin B12 deficiency:
Is B12 deficiency due to lack of intrinsic factor OR, normal intrinsic factor level, but not enough B12 absorption?
Autoimmune causes = pernicious anemia
Non-autoimmune causes = dietary, gastric bypass, tapeworm, pancreatic insufficiency
Folate deficiency: dietary or pregnancy causes
Other causes: alcohol use, drugs, malabsorption
Folate vs. B12 = The only difference is there are no neuro symptoms in folate deficiency
Macrocytic normochromic anemia
Caused by autoimmune disorder to attack against parietal cells
Lack of intrinsic factors
Intrinsic factor needed for B12 absorption → B12 deficiency
S&S: Neurological
Paresthesia, pins and needles in hands or feet
Lost sense of touch
Wobbly gait or trouble walking
Clumsiness and stiffness of the arms and legs
Dementia → long term impact
Diagnosis:
Antibodies against parietal cells
Macrocytic normochromic
Dietary intake related
Impacts rapidly dividing cells
Absorption of folate occurs in upper small intestine
Not dependent on any other factors
S&S:
Neuro symptoms not seen
Cheilosis, scales and fissures of the mouth
Stomatitis, painful ulcers of buccal mucosa and tongue
Diagnosis:
Methylmalonic acid and homocysteine level (B12 vs. folate deficiency)
Characterized by RBCs that are abnormally small and contain reduced amounts of hemoglobin; IRON DEFICIENCY
Disorders of iron metabolism, pophyrin and heme synthesis, and globin synthesis
Etiology:
Iron deficiency is the most commonly seen
Thalassemia is rare
Sideroblastic anemia is also rare
Anemia of chronic disease
Microcytic hypochromic
→ PATHOGENESIS:
Chronic blood loss, #1 common cause (pathologic vs. physiologic)
Menstruation (physiologic)
Occult blood loss (cannot see where blood loss is coming from, pathologic)
Malignancy (blood loss that you can see, pathologic)
Inadequate iron absorption (nutritional deficiency)
Diet low in heme iron (fish + meat)
Gastrointestinal disease or surgery (small intestine)
Excessive cow’s milk intake in infants (prevents iron absorption)
Pregnancy (physiologic demand increased)
Malabsorption
S&S: Impacts more of the epithelial
Symptoms of anemia plus;
Glossitis, smooth tongue
Restless legs
Angular stomatitis, cracking corners of the mouth
Koilonychia, thin brittle spoon-shaped fingernails
Tests for Iron Deficiency:
Hgb: ⬇️
Hct: ⬇️
MCV: ⬇️
Serrum ferritin: ⬇️
Serum iron/transferrin: ⬇️
Peripheral blood smear
Bone marrow iron stain (invasive and painful)
TREATMENT:
Oral iron salts; ferrous sulfate 325 mg po Q day (given to pregnant women)
Vitamin C can facilitate iron absorption or orange juice would suffice
DO NOT TRANSFUSE, UNLESS SEVERE (<6.5g/dl), considered aggressive form of treatment; will mask the issue
Acute blood loss
→ ETIOLOGY:
Hemolysis: sickle cell disease (appears in waves, not constant, 4-6 years can sickle), immune system, transfusion rejection (mismatched blood type)
Bone marrow suppression = myeloma
Anemia of chronic disease = chronic kidney disease
Hypothyroidism = slower production of RBCs
Normocytic anemia → microcytic hypochromic
Aka, anemia of inflammation
Decreased erythropoiesis (low erythropoietin)
Impaired iron utilization
INITIALLY normocytic-normochromic, but becomes microcytic-hypochromic
Treatment is aimed at an underlying disorder
Results from:
Decreased erythrocyte life span
Suppressed production of erythropoietin
Ineffective bone marrow response
Altered iron metabolism in macrophages
Lead to anemia of chronic disease
Hemorrhage: extraversion of blood from vessels
Hematoma: external or accumulation within a tissue
Benign (bruise) to fatal (retroperitoneal hematoma)
Jaundice
Large blood into cavities
Petechia, minute (1-2mm in diameter)
Purpura >3mm <1cm
Ecchymoses 1-2cm
→ Coagulopathy
Has 2 mechanisms
Platelet issue (dysfunction)
Coagulation cascade
→ Platelet Issue/Dysfunction
Increased bleeding time in presence of normal platelet counts
Disorders can be congential or acquired
TESTING FOR PLATELET DYSFUNCTION:
Order a coagulation panel
PT → Goes with PTT
PTT → Goes with PT, tests for the intrinsic or extrinsic pathways or the clotting factors
INR → Anticoagulate in the US that is universally understood to not withdraw care by external physicians in the states or overseas
→ COAGULATION PATHWAY DYSFUNCTIONS
Vitamin K deficiency, Hemophilia, DIC
Intrinsic, extrinsic, and common pathways
Autosomal dominant disorder
MOST COMMON inherited bleeding disorder
vWF facilitates platelet adhesion → causes mild or moderate bleeding
Defective platelet adherence PFA-100 test
→ Vitamin K deficiency (factors 2,7,9,10)
Necessary for synthesis and regulation of prothrombin (2), the procoagulant factors (7,9,10) and proteins C and S (anticoagulants)
Newborn/premature infants → liver not mature, get shot of vitamin K in bum right after birth
Poor intake → seen in homeless population and in addicts
Liver disease → seen with in combination with poor intake
Defective absorption:
Generalized malabsorption → Crohns and Celiacs Disease
Biliary disease
→ PATIENT RISK:
Diminished production by bacteria in gut (antibiotic treatment)
Vitamin K antagonists
Warfarin (Coumadin) → blood thinner, cannot eat vitamin K rich foods or else it will counteract the treatment
Heparin
Certain antibiotics
Inherited deficiency of factor 8 (hemophilia A) or factor 9 (hemophilia B) or factor 10 (hemophilia C)
Sex linked inheritance, almost all patients are males
Female carriers may have mild symptoms
Most bleeding into joints, muscles, mucosal and CNS bleeding uncommon
DIC
Develops RAPIDLY
Acquired disorder in which clotting and hemorrhage simultaneously occur
Endothelial damage/tissue factor is the primary initiator of DIC
→ PATHOPHYSIOLOGY:
Increased protease activity in the blood caused by unregulated release of thrombin with subsequent fibrin formation and accelerated fibrinolysis
Consumptive coagulopathy → uncontrolled hemostasis, patient bleeds out
→ ETIOLOGY:
High mortality rate
Infection by gram negative bacteria is the MOST COMMON CAUSE
Carcinomas/leukemias
Trauma, pregnancy (amniotic fluid gets into mother’s blood stream), transfusion reaction
Clinical signs and symptoms demonstrate wide variability:
Bleeding from venipuncture sites
Bleeding from arterial lines
Purpura, petechiae, and hemotomas
Symmetric cyanosis of the fingers and toes
Blood is a connective tissue
The blood is: 55% plasma, 45% formed elements (44% RBCs)
H & H: Hemoglobin and hematocrit, used for anemia testing
Hematocrit: % of any RBCs in any volume of blood (max is 44%)
CBC: Complete blood test; measures hemoglobin, hematocrit, platelet and WBC count, used for infection
ALWAYS ordered with a differential
“Inability of the blood to supply the tissues with adequate oxygen for proper metabolic function”
Either inadequate hemoglobin or RBC can lead to anemia
Hg: hemoglobin concentration
Hct: hematocrit concentration (packed cell volume)
RBC: red blood cell count
ANEMIA IS NOT A DISEASE; expression of an UNDERLYING disorder/disease
→ Normal Hg and Hct Levels
Hg < 13 in men; Hct <41%
Hg < 12 in women; Hct <36%
Hg <14 in men; Hg <12 in women (with malignancy)
Men | Women | |
---|---|---|
Hemoglobin (Hb) | 13.2-16.7 | 11.9-15.0 |
Hematocrit (Hct) | 38-48% | 35-44% |
Red Cell Count | 4.2-5.6 | 3.8-5.0 |
Reticulocyte Count | 0.5-1.5 | 0.5-1.5 |
Mean Cell Volume (MCV) | 81-97 | 81-97 |
Lastly, the anemia morphology:
Size: Macrocytic, microcytic, normocytic
Macro= the entire RBC
Micro= hemoglobin only
Hemoglobin content: Normochromic and hypochromic
Microcytic, MCV < 80 (reduced iron availability)
Normocytic, 80 < MCV < 100
Macrocytic, MCV > 100 (liver disease/B12 deficiency)
Hematocrit: volume of packed RBCs per unit of blood, expressed as a % (ex: 44% is the max)
38-44% is in normal range
Reticulocyte: young RBC, not functionally mature, anemia due to hemolysis or bleeding is characterized by reticulocytosis
Normal value: ~1%
Increase in reticulocytes is due to compensation to bring up the RBC
Mean corpuscular volume (MCV): reflect average size or volume of RBC, will tell if the patient is micro, macro, or normcytic
Mean cell hemoglobin concentration (MCHC): indicates the concentration of Hb in the average RBC, or the ratio of the weight of the Hb to the volume in which it is contained (chromicity/color)
Easy fatigue and loss of energy
Tachycardia, rapid heart rate
Shortness of breath
Headache
Difficulty concentrating
Dizziness
Pale skin
Leg cramps
Insomnia
Characterized by unusually large stem cells
Can also be termed megaloblastic anemias
→ PATHOGENESIS:
Defective DNA synthesis in rapidly dividing cells
Caused by B12 or folate deficiencies
Vitamin B12 deficiency:
Is B12 deficiency due to lack of intrinsic factor OR, normal intrinsic factor level, but not enough B12 absorption?
Autoimmune causes = pernicious anemia
Non-autoimmune causes = dietary, gastric bypass, tapeworm, pancreatic insufficiency
Folate deficiency: dietary or pregnancy causes
Other causes: alcohol use, drugs, malabsorption
Folate vs. B12 = The only difference is there are no neuro symptoms in folate deficiency
Macrocytic normochromic anemia
Caused by autoimmune disorder to attack against parietal cells
Lack of intrinsic factors
Intrinsic factor needed for B12 absorption → B12 deficiency
S&S: Neurological
Paresthesia, pins and needles in hands or feet
Lost sense of touch
Wobbly gait or trouble walking
Clumsiness and stiffness of the arms and legs
Dementia → long term impact
Diagnosis:
Antibodies against parietal cells
Macrocytic normochromic
Dietary intake related
Impacts rapidly dividing cells
Absorption of folate occurs in upper small intestine
Not dependent on any other factors
S&S:
Neuro symptoms not seen
Cheilosis, scales and fissures of the mouth
Stomatitis, painful ulcers of buccal mucosa and tongue
Diagnosis:
Methylmalonic acid and homocysteine level (B12 vs. folate deficiency)
Characterized by RBCs that are abnormally small and contain reduced amounts of hemoglobin; IRON DEFICIENCY
Disorders of iron metabolism, pophyrin and heme synthesis, and globin synthesis
Etiology:
Iron deficiency is the most commonly seen
Thalassemia is rare
Sideroblastic anemia is also rare
Anemia of chronic disease
Microcytic hypochromic
→ PATHOGENESIS:
Chronic blood loss, #1 common cause (pathologic vs. physiologic)
Menstruation (physiologic)
Occult blood loss (cannot see where blood loss is coming from, pathologic)
Malignancy (blood loss that you can see, pathologic)
Inadequate iron absorption (nutritional deficiency)
Diet low in heme iron (fish + meat)
Gastrointestinal disease or surgery (small intestine)
Excessive cow’s milk intake in infants (prevents iron absorption)
Pregnancy (physiologic demand increased)
Malabsorption
S&S: Impacts more of the epithelial
Symptoms of anemia plus;
Glossitis, smooth tongue
Restless legs
Angular stomatitis, cracking corners of the mouth
Koilonychia, thin brittle spoon-shaped fingernails
Tests for Iron Deficiency:
Hgb: ⬇️
Hct: ⬇️
MCV: ⬇️
Serrum ferritin: ⬇️
Serum iron/transferrin: ⬇️
Peripheral blood smear
Bone marrow iron stain (invasive and painful)
TREATMENT:
Oral iron salts; ferrous sulfate 325 mg po Q day (given to pregnant women)
Vitamin C can facilitate iron absorption or orange juice would suffice
DO NOT TRANSFUSE, UNLESS SEVERE (<6.5g/dl), considered aggressive form of treatment; will mask the issue
Acute blood loss
→ ETIOLOGY:
Hemolysis: sickle cell disease (appears in waves, not constant, 4-6 years can sickle), immune system, transfusion rejection (mismatched blood type)
Bone marrow suppression = myeloma
Anemia of chronic disease = chronic kidney disease
Hypothyroidism = slower production of RBCs
Normocytic anemia → microcytic hypochromic
Aka, anemia of inflammation
Decreased erythropoiesis (low erythropoietin)
Impaired iron utilization
INITIALLY normocytic-normochromic, but becomes microcytic-hypochromic
Treatment is aimed at an underlying disorder
Results from:
Decreased erythrocyte life span
Suppressed production of erythropoietin
Ineffective bone marrow response
Altered iron metabolism in macrophages
Lead to anemia of chronic disease
Hemorrhage: extraversion of blood from vessels
Hematoma: external or accumulation within a tissue
Benign (bruise) to fatal (retroperitoneal hematoma)
Jaundice
Large blood into cavities
Petechia, minute (1-2mm in diameter)
Purpura >3mm <1cm
Ecchymoses 1-2cm
→ Coagulopathy
Has 2 mechanisms
Platelet issue (dysfunction)
Coagulation cascade
→ Platelet Issue/Dysfunction
Increased bleeding time in presence of normal platelet counts
Disorders can be congential or acquired
TESTING FOR PLATELET DYSFUNCTION:
Order a coagulation panel
PT → Goes with PTT
PTT → Goes with PT, tests for the intrinsic or extrinsic pathways or the clotting factors
INR → Anticoagulate in the US that is universally understood to not withdraw care by external physicians in the states or overseas
→ COAGULATION PATHWAY DYSFUNCTIONS
Vitamin K deficiency, Hemophilia, DIC
Intrinsic, extrinsic, and common pathways
Autosomal dominant disorder
MOST COMMON inherited bleeding disorder
vWF facilitates platelet adhesion → causes mild or moderate bleeding
Defective platelet adherence PFA-100 test
→ Vitamin K deficiency (factors 2,7,9,10)
Necessary for synthesis and regulation of prothrombin (2), the procoagulant factors (7,9,10) and proteins C and S (anticoagulants)
Newborn/premature infants → liver not mature, get shot of vitamin K in bum right after birth
Poor intake → seen in homeless population and in addicts
Liver disease → seen with in combination with poor intake
Defective absorption:
Generalized malabsorption → Crohns and Celiacs Disease
Biliary disease
→ PATIENT RISK:
Diminished production by bacteria in gut (antibiotic treatment)
Vitamin K antagonists
Warfarin (Coumadin) → blood thinner, cannot eat vitamin K rich foods or else it will counteract the treatment
Heparin
Certain antibiotics
Inherited deficiency of factor 8 (hemophilia A) or factor 9 (hemophilia B) or factor 10 (hemophilia C)
Sex linked inheritance, almost all patients are males
Female carriers may have mild symptoms
Most bleeding into joints, muscles, mucosal and CNS bleeding uncommon
DIC
Develops RAPIDLY
Acquired disorder in which clotting and hemorrhage simultaneously occur
Endothelial damage/tissue factor is the primary initiator of DIC
→ PATHOPHYSIOLOGY:
Increased protease activity in the blood caused by unregulated release of thrombin with subsequent fibrin formation and accelerated fibrinolysis
Consumptive coagulopathy → uncontrolled hemostasis, patient bleeds out
→ ETIOLOGY:
High mortality rate
Infection by gram negative bacteria is the MOST COMMON CAUSE
Carcinomas/leukemias
Trauma, pregnancy (amniotic fluid gets into mother’s blood stream), transfusion reaction
Clinical signs and symptoms demonstrate wide variability:
Bleeding from venipuncture sites
Bleeding from arterial lines
Purpura, petechiae, and hemotomas
Symmetric cyanosis of the fingers and toes