Hematology

anemia

• reduction in the total number of erythrocytes in the circulating blood or in the quality or quantity of hemoglobin

• causes include:

  • impaired erythrocyte production

  • acute or chronic blood loss

  • increased erythrocyte destruction

  • combination of the above

classifying anemia

• size → “-cytic”

  • macrocytic, microcytic, normocytic

• hemoglobin content → "-chromic"

  • normochromic

  • hypochromic

• anisocytosis

  • red cells are present in various sizes

• poikilocytosis

  • red cells are present in various shapes

anemia symptoms

• physiologic manifestations:

  • reduced oxygen-carrying capacity-hypoxemia

• variable symptoms based on severity and the ability for the body to compensate

• classic findings:

  • faituge

  • weakness

  • dyspnea

  • pallor

• other findings:

  • yellowing eyes & skin

  • pale & cold skin

  • hypotension

  • palpitations

  • tachycardia

  • spleen enlargement

• in severe cases:

  • fainting

  • chest pain

  • angina

  • heart attack

pernicious anemia

• caused by a lack of intrinsic factor from the gastric parietal cells

  • required for vitamin B12 absorption

  • results in vitamin B12 deficiency

  • unable to make healthy red blood cells

• includes typical anemia symptoms, but also:

  • nerve demyelination

  • absence of intrinsic factor

  • loss of appetite

  • abdominal pain

  • beefy red tongue (atrophic glossitis)

  • icterus

  • splenic enlargement

• causes a B12 deficiency — there are other causes of this tho

risk factors for pernicious anemia

• family hx

• Northern European descent

• chronic gastritis or gastric surgery

• medications

• vegetarian diet

tx of pernicious anemia

• vitamin B12 (cyanocobalamin)

  • needs to be larger doses if PO

  • parenteral administration

    • typically given IM, but sometimes deep SQ

    • sometimes nasally

• also found in food, but not enough to treat it:

  • fish

  • milk

  • eggs

  • some cheese

  • fortified cereals

• B12 deficiency without an intrinsic factor deficiency? Give PO

folate deficiency anemia

• absorption of folate occurs in the upper small intestine — if they have Crohns its a problem

• not dependent on any other factor

• similar symptoms to pernicious anemia except neurological manifestations generally not seen

• what if we don’t have it?

  • neuro tube defects in fetuses

tx of folate deficiency anemia

• daily PO doses

• also found in food, so most people aren’t deficient unless absorption issue:

  • green veggies

  • citrus

  • beans

  • bread

  • cereal

  • pasta

• NOTE - can potentially mask symptoms of pernicious anemia, so need to rule that out first

nursing implications for folic acid

• give w/ food

• folic acid may also be given IV and added to TPN solutions

• monitor for therapeutic response:

  • improved nutritional status

  • increased weight, activity tolerance, well-being

  • absence of fatigue

• Note - have to check both B12 and folate levels because symptoms are so similar

iron deficiency anemia

• most common type of anemia

• nutritional iron deficiency

• metabolic or functional deficiency

• manifestations when serum Hgb decreased to 7 or 8 gm/dl

  • early: fatigue, weakness, SOB, pale ear lobes, palms, conjunctiva

  • progression causes:

    • brittle, thin, coarsely ridged, and spoon shaped nails (koilonychia)

    • a red, sore, and painful tongue

    • dry, sore corners of mouth (angular stomatitis)

causes of iron deficiency anemia

• excessive menses

• GI bleed

• excessive need (pregnancy)

• nutritional deficiency

tx of iron deficiency anemia

• iron supplements

  • can be PO or IV

• certain foods enhance absorption:

  • OJ (vit C)

  • fish

• note → when treating it, look for the cause!

  • GI bleeds

iron treatments

• may be given as a single drug or as part of a multivitamin preparation

• oral iron preparations are available as ferrous salts

  • ferrous fumarate, ferrous gluconate, ferrous sulfate

• parenteral

  • iron dextran

  • iron sucrose

  • ferric gluconate

  • ferumoxytol

adverse effects of iron

• most common cause of pediatric poisoning deaths

• causes:

  • n/v

  • constipation

  • stomach cramps and pain

  • sometimes diarrhea instead

• causes black, tarry stools

• liquid oral preparations may stain teeth

• injectable forms cause pain upon injection

iron toxicity

• symptomatic & supportive measures

  • suction & maintenance of the airway

  • correction of acidosis

  • control of shock & dehydration w/ IV fluids or blood

  • oxygen

  • vasopressors

• in pts w/ severe symptoms of iron intoxication, such as coma, shock, or seizures, chelation therapy w/ deferoxamine is initiated

  • deferiprone is otherwise used in iron overload

iron dextran

• may cause anaphylactic reactions, including major orthostatic hypotension and fatal anaphylaxis

• a test of 25mg of iron dextran is administered before injection of the full dose, and then the remainder of dose is given after 1 hour

• used less frequently now; replaced by newer products like ferric gluconate & iron sucrose

ferric gluconate

• indicated for repletion of total body iron content in pts with iron-deficiency anemia who are undergoing hemodialysis

• risk of anaphylaxis is much less than with iron dextran, and a test dose is not required

• doses higher than 125mg are associated with increased adverse events, including:

  • abdominal pain

  • dyspnea

  • cramps

  • itching

iron nursing implications: general assessments

• assess pt hx and medication hx, including drug allergies & history of reaction to iron

• assess baseline lab value, especially hgb, hct, and reticulocytes

• obtain nutritional assessment

• ferrous salts are contraindicated in pts w/ UC, PUD, liver disease, and other GI disorders (would further irritate gastric mucosa)

iron nursing implications: PO doses

• might have to dilute it (follow instructions)

• instruct the pt to take liquid iron preparations through a straw to avoid staining tooth enamel

• oral forms of iron should be taken between meals for maximum absorption, but may be taken with meals if GI distress occurs

• oral forms should be given w. juice but not w/ milk or antacids

• pts should remain upright for 15 to 30 minutes after oral iron doses to avoid esophageal corrosion

• pts should be encouraged to eat foods high in iron and folic acid

nursing implications for iron dextran

• have resuscitative equipment available in case of an anaphylactic reaction

• iron dextran is contraindicated in all anemias except for iron-deficiency anemia

sideroblastic anemia

• group of disorders characterized by anemia

• acquired (idiopathic), hereditary, reversible

  • acquired? → vitamin B6 deficiency from not enough dietary intake, chronic alcohol abuse, or isoniazid

• altered mitochondrial metabolism causing ineffective iron uptake & resulting in dysfunctional hemoglobin synthesis (not an iron deficiency)

• ringed sideroblasts within the bone marrow are diagnostic

  • sideroblasts are erythroblasts that contain iron granules that have not been synthesized into hemoglobin

• manifestations:

  • common anemia signs/symptoms

  • diagnostic: hemosiderosis & hemochromatosis (iron overload)

    • body is absorbing too much iron from the food we eat

    • tx: remove some blood

aplastic anemia

• can cause pancytopenia

• pure red cell aplasia (bone marrow isn’t making enough RBCs)

• fanconi anemia (genetic disorder that can cause aplastic anemia)

hemolytic anemia

• accelerated destruction of red blood cells (being destroyed faster than the body is making new ones)

  • autoimmune hemolytic anemias (your body)

  • drug-induced hemolytic anemia

    • some abx, NSAIDs, high doses of tylenol

anemia of chronic inflammation/disease

• mild to moderate anemia seen in:

  • AIDS

  • RA

  • lupus

  • hepatitis

  • renal failure

  • malignancies

• pathologic mechanisms:

  • decreased erythrocyte life span

  • ineffective bone marrow response to erythropoietin

  • altered iron metabolism

epogen (epoetin alfa)

• biosynthetic form of natural EPO

• used for tx of anemia associated w/ end-stage renal disease, chemo, and zidovudine therapy

• still need iron - might also need replaced

• longer acting form of epoetin called darbepoetin (aranesp)

• contraindications:

  • drug allergy

  • uncontrolled hypertension

  • hemoglobin >10 g/dL

  • risk of thrombosis

• most frequent AEs:

  • hypertension

  • fever

  • headache

  • pruritus & rash

  • arthralgia

  • injection site reaction

Rh factor

• D-antigen is the base for Rh + & -, it’s on the RBC

• absence of D-antigen = Rh negative

• presence of D-antigen = Rh positive

pneumonic for febrile nonhemolytic transfusion reactions

H - hemolysis (acute or delayed hemolytic transfusion reaction)

A - allergy (anaphylactic transfusion reactions)

L - lung (transfusion-related acute lung injury… TRALI)

O - overload (transfusion-associated circulatory overload… TACO)

polycythemia

• overproduction of RBCs

relative polycythemia

• result of dehydration

• fluid loss results in relative increases of red cell counts and Hgb and Hct values

absolute polycythemia

• primary absolute

  • abnormalities of stem cells in the bone marrow

  • polycythemia vera (PV)

• secondary absolute

  • increase in EPO as a normal response to chronic hypoxia or an inappropriate response to EPO-secreting tumors

polycythemia vera

• slow growing blood cancer → overproduction of RBCs

• increases risk of clots

• rare in children

• men 2x more likely to have it

• first sign that something is wrong → get painful itching from heat or water

• S&S:

  • fatigue

  • headache

  • dizziness

  • episodic blurred vision

  • peripheral tingling, burning, or itching

  • hypertension

  • mucosal cyanosis

  • bruising

  • petechiae

  • enlarged spleen or liver

leukocytosis

• higher WBC count than normal

• a normal protective response to physiologic stressors

leukopenia

• never normal or beneficial

• low WBC count that predisposes pt to infections

granulocytosis

• AKA neutrophilia

• normal in early infection

• if the need for neutrophils increases beyond the supply, immature neutrophils (banded neutrophils) are released into the blood

neutropenia

• AKA granulocytopenia

• or agranulocytosis (complete absence of WBCs)

  • if became septic, would die in 3-5 days

• reduction in circulating neutrophils

• causes:

  • prolonged severe infection (production can’t keep up w/ demand)

  • decreased production

  • reduced survival

  • abnormal neutrophil distribution and sequestration

  • interference w/ hematopoiesis

  • immune mechanisms

  • chemotherapy destruction

  • ionizing radiation

lymphocytosis

• elevated lymphocytes (B, T, NK cells)

• acute viral infections

  • epstein-barr virus

lymphocytopenia

• low lymphocyte count

• immune deficiencies

• drug destruction

• viral destruction

infectious mononucleosis

• AKA “mono”

• transmission:

  • most common in young people

  • less than 25 y/o

  • predominantly transmitted via saliva

  • known as the kissing disease

• causes:

  • Epstein-Barr virus

• S&S:

  • faituge

  • decreased energy

  • sore throat (severe)

  • tonsils enlarged & reddeded

  • fever

  • headache

  • skin rash

  • swollen lymph glands

  • pain in LUQ - splenomegaly

  • loss of appetite

• Treatment:

  • rest

  • throat soothing measures

  • acetaminophen/ibuprofen

  • low energy/impact activity

  • gradual increase in activity

  • course is self-limiting

• diagnostics:

  • mono spot (herophil agglutination test)

  • clinical symptoms

  • cytomegalovirus can mimic mono symptoms

infectious mononucleosis pt.2

• acute, self-limiting infection of B lymphocytes transmitted by saliva through personal contact

• commonly caused by the epstein-barr virus (EBV) — 85%

  • B cells have an EBV receptor

  • other viral agents resembling IM inlclude CMV, hepatitis, flu, HIV

• symptoms:

  • fever

  • sore throat

  • swollen cervical lymph nodes

  • increased lymphocyte count

  • atypical (activate) lymphocytes

  • serious complications are infrequent (<5%) - splenic rupture

• >50% lymphocytes and at least 10% atypical lymphocytes

• tx: symptomatic

leukemias

• malignant disorder of the blood and blood-forming organs

• excessive accumulation of leukemic cells

• pancytopenia if cells crowd bone marrow

• acute leukemia:

  • presence of undifferentiated or immature cells, usually blast cells

  • acute lymphocytic leukemia (ALL)

  • acute myelogenous leukemia (AML)

• chronic leukemia

  • predominant cell is mature but does not function normally

  • chronic myelogenous leukemia (CML)

  • chronic lymphocytic leukemia (CLL)

S&S of leukemias

• anemia

• bleeding

• purpura

• petechiae

• ecchymosis

• thrombosis

• hemorrhage

• DIC

• infection

• weight loss

• bone pain

• elevated uric acid levels

• liver, spleen, and lymph node enlargement

  Pneuomonic: ANT

• A = anemia

• N = neutropenia

• T = thrombocytopenia
  

acute lymphoblastic leukemia

• most common pediatric cancer

• S&S:

  • bleeding

  • infection

  • lymphadenopathy

  • fever

  • bone pain

leukemia in kids

• most common malignancy of childhood

• 80% to 85% are acute lymphoblastic leukemias (ALL); remainder acute myelogenous leukemia (AML)

• result of ecogenetics:

  • down syndrome risk factor

• manifestions:

  • pallor

  • fatigue

  • purpura

  • fever

• blast cell is hallmark of acute leukemia

• 5-year survival rate is 80%

lymphadenopathy

• enlarged lymph nodes that become palpable and tender

• local lymphadenopathy:

  • drainage of an inflammatory lesion located near the enlarged node

• general lymphadenopathy:

  • occurs in the presence of malignant or nonmalignant disease

• painless lumps = very concerning

lymphomas

• two types (non-hodgkin, and hodgkin)

• malignancy of lymphocytes (B, T, and NK cells)

• usually involves lymph nodes, tonsils, and spleen

• most common hematological malignancy in adults

hodgkin lymphoma

• more common in men

• malignant transformation of a lymphocyte and proliferation of lymphocytes, histiocytes, their precursors, and derivatives in lymphoid tissues

• characterized by the presence of Reed-Sternberg cells in the lymph nodes

  • Reed-Sternberg cells are necessary for diagnosis, but they are not specific to Hodgkin lymphoma

• S&S:

  • adenopathy

  • mediastinal mass

  • splenomegaly

  • abdominal mass

  • fever

  • weight loss

  • night sweats

  • pruritus

• laboratory findings:

  • thrombocytosis

  • leukocytosis

  • eosinophilia

  • elevated ESR

  • elevated alkaline phosphatase

  • paraneoplastic syndromes

non-hodgkin lymphomas

• generic term for a diverse group of lymphomas

• non-hodgkin lymphomas are linked to chromosome translocations, viral & bacterial infections, environmental agents, immunodeficiencies, and autoimmune disorders

  • also AIDS, HIV, and H.pylori

non-hodgkin lymphoma (NHL) in kids

• nodular or diffuse (most in childhood are diffuse)

• childhood NHL subdivided

  • large cell (histiocytic)

  • lymphoblastic

  • small noncleaved cell (burkitt or non-burkitt)

• etiology viral, chronic immunostimulation, congenital immunodeficiency syndromes, and genetics

• manifestations specific to site involved

• mediastinal mass

• tx of chemo and radiation

• 60% to 80% cure rate

burkitt lymphoma

• most common type of non-hodgkin lymphoma in children

• burkitt lymphoma is a very fast-growing tumor of the jaw and facial bones

  • epstein-barr virus is found in nasopharyngeal secretions of pts

multiple myeloma (MM)

• malignant proliferation of plasma cells

• infiltrate bone marrow and aggregate into tumor masses in skeletal system

• M-proteins

• Bence Jones protein

• hypercalcemia, renal failure, and bone lesions

• S&S:

  • hypercalcemia

  • renal failure

  • anemia

  • bone pain

alterations in splenic function

• splenomegaly

• hypersplenism

• tx: splenectomy

thrombocytopenia

• platelet count >150,000/mm³ (low)

• causes:

  • hypersplenism

  • autoimmune disease - ITP and TTP

  • hypothermia

  • viral or bacterial infections that cause disseminated intravascular coagulation (DIC)

  • HIT

primary thrombocythemia (thrombocytosis)

• platelet count >600,000/mm³ (high)

• myeloproliferative disorder of platelet precursor cells

  • megakaryocytes in the bone marrow are produced in excess

• microvascular thrombosis occurs

immune thrombocytopenic purpura (ITP)

• immune (idiopathic) thrombocytopenic purpura (ITP)

  • IgG antibody that targets platelet glycoproteins

  • antibody-coated platelets are sequestered and removed from the circulation

  • the acute form of ITP that often develops after a viral infection is one of the most common childhood bleeding disorders

  • manifestions:

    • petechiae and purpura

    • progressing to major hemorrhage

  • this is pretty common

disseminated intravascular coagulation (DIC)

• how is this different from TTP?

• in TTP, the clots have a lot of von Willebrand factor

• in DIC, the clots are mostly fibrin

• rare but life threatening

• accelerated clotting within blood vessels → increased consumption of platelets & clotting factor → uncontrollable bleeding

vitamin K deficiency

• vitamin K is necessary for synthesis and regulation of prothrombin, the prothrombin factors (II, VII, XI, X), and proteins C and S (anticoagulants)

• a deficiency increases bleeding time

alterations of coagulation in liver disease

• causes a broad range of hemostasis disorders:

  • defects in coagulation

  • fibrinolysis

  • platelet number and function

thrombotic thrombocytopenic purpura (TTP)

• a thrombotic microangiopathy

  • platelets aggregate, form microthrombi, and cause occlusion of arterioles and capillaries

• this one isn’t common

• von willdebrand factor in clots