n255c hematologic disorders

hematologic system

• consists of the blood and bone marrow

• blood:

  • RBCs

  • WBCs

  • platelets

  • plasma

• involved in:

  • O2 and CO2 transport

  • coagulation

  • combating infections

erythrocytes: RBCs

• function in body:

  • transport O2 and CO2 to tissues → like bus transporting ppl

  • buffer for acid-base balance → likely to become acidic if no RBCs

erythropoiesis

• creation/production of RBCs

• signaled by erythropoietin (EPO)

  • produced by kidneys when tissues have low oxygenation

  • erythropoietin tells stem cells to turn bone marrow into RBCs

• problem w kidneys = decreased erythropoietin = less production of RBCs

  • can give artificial erythropoietin to ppl w kidney probs

• requires nutrients → iron, folic acid, vitamin B12

hemolysis

• RBCs are destroyed

• can cause high K+ bc all intraceullar contents spill out

anemia

• deficiency in RBCs

• caused by:

  • blood loss → surgery/trauma

  • decreased production of RBCs → kidney failure/nutrient deficiency

  • increased destruction → hemolysis > erythropoiesis

• diagnosed by:

  • CBC

  • investigation of cause

• classified by morphology or etiology

• risk factors:

  • age → elderly and younger ppl

  • sex → females of childbearing age who are menstruating

  • chronic diseases → chronic kidney or liver disease

  • blood loss

  • genetics → sickle cell anemia

  • diet deficiencies → lacking in folic acid, iron, B12

  • excessive ETOH intake → gastric irritant, prevents nutrient absorption

  • chemotherapy & radiation → bone marrow suppression

  • exposure to toxic agents

  • low socioeconomic status → nutrient deficiencies

RBC lab studies

• hemoglobin (Hgb) → what binds O2 in our RBCs

  • female: 11.7-16.9 g/dL

  • male: 13.2 - 17.3 g/dL

  • critically low is <7.. im scared.. lower than 7 = going to heaven

• hematocrit (Hct) → % of blood that are RBCs

  • female: 35-47%

  • male: 39-50%

  • critically low is 21%

anemia: clinical manifestations

• FATIGUE, WEAKNESS

  • less O2 carrying capacity = more hypoxemic = less O2 in cells = tired

• pallor

  • oxygenated RBCs give blood red color

  • severe anemia = hypoxic state = blood shifts to vital organs

• SOB, tachycardia

  • body breathes harder and faster to try to get more O2 in and pump it to tissues

• cool skin/cold intolerance

  • shifting of blood to vital organs instead of extremities

• dizziness, HA

• S/S of hypoxia

• **anemic pts can have normal SpO2… O2 is binding to hemoglobin/RBCs they have, but they don’t actually have that many RBCs..

iron-deficient anemia

• etiology

  • inadequate intake

  • increased need

  • malabsorption

  • blood loss

  • hemolysis

• general manifestations

  • brittle, thin, spoon-shaped nails → iron is needed for epithelial creation

  • pica → craving things that have no nutritional value

  • glossitis & cheilitis → inflammation of tongue and lips

• diagnostics

  • run iron panel

  • decreased Hgb, Hct, RBCs, reticulocytes, serum iron, ferritin

    • ferritin is stores form of iron

  • increased transferrin and total iron binding capacity (TIBC)

    • transferrin moves iron around

  • decreased microcytic and hypochromic RBCs → MCV, MCH, MCHC

• iron supplements

  • PO: ferrous sulfate/ferrous gluconate

    • take with vitamin C on an empty stomach for better absorption

    • SE: constipation, GI upset, diarrhea, staining teeth, black stools (watch out and differentiate between GI bleed)

  • parenteral iron (IM or IV)

  • educate:

    • encourage fluid intake and fiber

    • avoid antacids and calcium supplements

    • adherence → keep taking even if feeling better

• management

  • diet modifications = red meat, poultry, fish, leafy greens, beans, legumes, whole grain/enriched bread and cereals

vitamin B12 deficiency anemia/pernicious anemia

• etioogy

  • inadequate dietary intake

  • pernicious anemia

    • autoimmune → body attacks intrinsic factor that usually helps to absorb B12

  • malabsorption syndromes

  • gastric surgery

  • alcoholism

  • medications

• manifestations

  • glossitis/cheilitis

  • neurologic symptoms = need B12 for myelin and surrounding of nerves for transduction = tingling/numbness

• diagnostics

  • decreased cobalamin (determines B12 level)

  • decreased Hgb, Hct, RBC, reticulocyte count

  • megaloblastic RBCs (increased MCV)

  • intrinsic factor antibodies

  • neurologic symptoms

• management

  • PO supplementation/dietary modification

  • IM B12 injfections

  • monitor for neuro symptoms

  • safety (falls, burns, trauma)

  • oral care → glossitis/cheilitis

folic acid deficiency anemia

• etiology

  • inadequate intake

  • pregnancy (increased demand) → prenatals rich in folic acid!

  • malabsorption, gastric surgery

  • alcoholism

• general manifestations

  • glossitis/cheilitis

• diagnostics

  • decreased folate

  • normal cobalamin

  • decreased Hgb, Hct, RBC, reticulocyte count

  • megaloblastic RBCs (increased MCV)

• management

  • PO folic acid replacement

  • diet → dark leafy greens, enriched products, orange juice, peanuts, avocado

hemolytic anemia

• destruction of RBCs faster than production

• etiology

  • hereditary defects within RBCs (sickle cell)

  • external factors: physical destruction, autoimmune, transfusion reaction, infections, drug-induced

• diagnostics

  • decreased Hgb, Hct, RBC

  • increased bilirubin

    • waste product made when destroying RBCs

    • usually liver handles it by turning into bile and excreting

    • too much hemolysis here so it builds up

• clinical manifestations

  • general anemia symptoms

  • jaundice

    • eyes and yellow skin tint

  • pruritis

    • bilirubin irritating to skin = itching

sickle cell disease

• genetic disorder causing abnormal Hgb in RBCs

• screen in ALL people

• 1 in 365 AA births are affected by SCD

  • just more common in african americans

• neonate screening

• both parents have to have sickle cell trait

• sickle RBCs are rigid, sticky, and prone to clumping → leads to vascular blockages

  • kidney bean shape RBCs and no longer effective in carrying O2

• clinical manifestations

  • SEVERE PAIN in back, chest, extremities, abdomen

  • vaso-occlusive crisis AKA sickle cell crisis d/t blockage of blood flow and lack of O2

    • need opioids and will need high doses

    • triggers = infection, high altitude, stress, surgery, dehydration, cold

  • complications:

    • stroke, heart failure, kidney failure, infections (blood block to spleen), acute chest syndrome (blood block to lungs = death)

• management

  • treat the trigger

  • pain management w NSAIDs/opioids

  • rest

  • O2

  • heat therapy

  • IV fluids → ENCOURAGE FLUIDS to dilute blood and prevent sticking

  • hydroxyurea → decreases sickling of RBCs

    • SE of bone marrow suppression so less sickled RBCs are created

    • avoid if pregnant

  • blood transfusion

    • if Hgb <7

  • hematopoietic stem cell transplant (HSCT)

    • need to be qualified and have proper donor

• patient education

  • understanding disease process/triggers

  • vaccinations/infection prevention

  • pain management techniques

polycythemia

• too many RBCs → hyperviscosity

• diagnostics:

  • increased Hgb, Hct, RBCs

  • EPO: decreased with primary, increased with secondary

  • increased WBC and PLT (primary polycythemia) bone marrow biopsy

• clinical manifestations:

  • HTN → d/t thicker blood and higher pressure needed to pump

  • HA, dizziness → from HTN

  • reddish skin → more RBCs = more color

  • pruritis → irritating

  • THROMBOSIS

• treatments:

  • phlebotomy → drain excess RBCs

  • IV fluids → to prevent thrombosis

  • stay hydrated

  • bone marrow suppressants → hydroxyurea

  • low-dose aspirin → combat clotting

  • patient education → stop smoking, avoid high altitudes, prevent clots

thrombocytes (platelets)

• initiate the clotting process by forming a platelet plug at the site of injury

• platelets produced in bone marrow

  • Thrombopoietin comes from liver → to prompt creation of platelets

  • liver problems = platelet issues

• regulated by the thrombopoietin (TPO) from the liver

• 80% of platelets circulate, 20% are stored in spleen

• PLATELETS (PLT): 150K - 400K/microliter

  • <150k = thrombocytopenia d/t decreased production or increased destruction

thrombocytopenia

• reduction of platelets = <150k/microliter

• decreased production:

  • bone marrow suppression

  • aplastic anemia: low platelets, RBCs, and WBCs

  • chemotherapy or radiation

  • cirrhosis = liver failure

• increased destruction:

  • immune thrombocytopenic purpura (ITP)

    • autoimmune disorder where body attacks its own platelets

    • severe thrombocytopenia and high risk for bleeding

  • heparin-induced thrombocytopenia & thrombosis syndrome (HITTS)

    • heparin doesn’t normally impact platelets

    • doesn’t happen immmediately → takes 5-7 days of heparin therapy

    • platelet levels start to drop drop drop

• clinical manifestations:

  • BLEEDING

  • epistaxis, gingival bleeding

  • menorrhagia → heavy periods

  • melena stools

  • petechiae

  • purpura → larger and bigger red dots

  • ecchymosis → big bruises

  • hematuria

  • hemoptysis → coughing up blood

• management:

  • monitor for S/S of bleeding

  • avoid IM, SQ injections

  • soft toothbrush and electric razor

  • fall prevention

  • avoid NSAIDs, anticoags, antiplatelets

  • avoid contact sports

  • transfuse platelets if <10k/microliter

  • ITP:

    • give steroids to suppress immune system so body stops destroying platelets

    • IVIG

    • immunosuppressants

    • splenectomy

  • HITTS:

    • stop heparin immediately

    • alternative anticoags