HEMATOLOGIC DISORDERS Flashcards by michaela gail

Most common hematologic condition. Reflects a fewer than normal number of erythrocytes (RBCs).
Not a specific disease but a SIGN of an underlying disorder. If untreated, may progress to TISSUE HYPOXIA.

anemia

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Three-way compensatory mechanisms:

Increased HR & RR
Redistributing blood to the brain and heart
Increase erythropoietin production by the kidneys

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general s/sx of anemia

yellowing of eyes, skin
pale, cold skin
SOB
weakness
changed stool
fatigue, dizziness, fainting
low blood
palpitations, high PR
chest pain, angina, heart attack
spleen enlargement

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anemia goals

decrease fatigue
adequate nutrition
adequate tissue perfusion
absence of complications

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Defective DNA Synthesis

Vitamin B12 deficiency

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Decreased Hemoglobin Synthesis

Iron deficiency
Thalassemia

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Decreased # of Erythrocyte Precursors

Aplastic

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Intrinsic (Hemolytic)

Sickle cell

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Extrinsic

Physical trauma
Antibodies
Infectious agents
Toxins

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loss of blood - Acute

Trauma
Blood vessel rupture

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loss of blood - chronic

Gastritis
Hemorrhoids
Menstruation

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A significant decrease in RBC production by the bone marrow.

Decreased RBC is reflected as low or inappropriately normal reticulocyte count

Results from marrow damage d/t medications, chemicals, or lack of factors in the RBC formation.

Hypoproliferative Anemia

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Hypoproliferative Anemia Most common types:

Iron-deficiency
Pernicious
Folic acid deficiency
Aplastic

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Most common type of anemia in all age group

Occurs when dietary iron intake is inadequate for hemoglobin synthesis.

Most common cause is bleeding (ulcers, gastritis, IBD, or GI tumors, and menorrhagia)

Iron Deficiency Anemia

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Iron Deficiency Anemia Hallmark Signs and Symptoms

pica
atrophic glossitis
angular cheilitis
koilonychia

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for iron deficiency anemia:

Exogenous erythropoietin
Given for CKD patients undergoing hemodialysis and/or chemotherapy
Increases hemoglobin and hematocrit levels, reducing RBC transfusions.

Epoetin Alfa (Epogen)

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Epoetin Alfa (Epogen) WOF

HTN
joint / muscle pain
diarrhea
cold

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A type of megaloblastic anemia (abnormally large, immature RBCs which are oval-shaped).

Directly attributed from an insufficient supply of folate (vitamin B9) within the span of 4 months.

Has the same signs and symptoms of pernicious anemia with the absence of neurologic problems. It distinguishes the two types of megaloblastic anemia.

Folic Acid Deficiency Anemia

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Folic Acid Deficiency Anemia
Hallmark Signs and Symptoms

gray hair
diarrhea
tiredness
cracked lips

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A type of megaloblastic anemia (abnormally large, immature RBCs which are large and oval-shaped).

Inability to absorb Vitamin B12 in the GI tract d/t lack of intrinsic factor

Damage to the parietal cells, decreases secretion of HCl and inhibits the secretion of the intrinsic factor, which also affects folate synthesis in the body.

Pernicious Anemia

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common causes of pernicious anemia

Absence of intrinsic factor in the parietal cells of the GI mucosa
GI mucosal atrophy, autoimmune destruction of the parietal cells
GI surgery

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Pernicious Anemia
Hallmark Signs and Symptoms

wt loss
loss of appetite
NV, abd distention, diarrhea, constipation
paresthesia, poor gait
memory loss, depression

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mgmt for pernicious anemia

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vitamin b12
iron
folate
cobalamin
pyridoxine

Characterized by bone marrow hypoplasia or aplasia resulting to pancytopenia.

Pancytopenia is a decrease of all blood cell types (RBC, WBC, and platelets)

Caused by viral infections, pregnancy, medications, chemicals, exposure to radiation agents like benzene.

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aplastic anemia

Destruction or damage to hematopoietic stem cells in the bone marrow, leading to decreased production of all blood cell lines.

Aplastic Anemia

s/sx 3 phases aplastic anemia

anemic - pallor, fatigue, dizziness neutropenic - fever, malaise, diarrhea, adventitious BS thrombocytopenic - bleeding, petechiae, ecchymoses

aplastic anemia mgmt

Immunosuppressive therapy: Corticosteroids Cyclosprine Cytoxan Antihymocyte globulin Antilymphocyte globulin

RBCs are destroyed faster than the bone marrow can produce, which cause tissue hypoxia. Triggers kidney to produce erythropoietin, which let the bone marrow prematurely release new RBCs. Conclusive laboratory: increased reticulocyte count

hemolytic anemia

An inherited, severe form of hemolytic anemia, which results from inheritance of the sickle hemoglobin gene. Sickle cell hemoglobin (HbS) is formed when exposed to low oxygen tension (PPO2). Red blood cells to become rigid, sticky, and shaped like sickles or crescent moons.

sickle cell anemia

complications of sickle cell anemia

thrombosis pulmo HTN, pneumonia hepatomegaly renal failure retinopathy, blindness HF splenic atrophy stasis ulcers of hands and feet

mgmt of sickle cell

Hydroxyurea (used to increase fetal hemoglobin during pregnancy) Splenectomy, if recurrent splenic sequestration is present

A group of inherited blood disorders characterized by reduced or absent production of one or more globin chains that make up hemoglobin.

thalassemia

thalassemia: Milder form without significant symptoms

alpha

thalassemia: Most prevalent and can have a mild-to-severe form

beta

Characterized as both beta-globin genes are severely affected or non-functional, leading to absent or severely reduced beta-globin production. Develops severe anemia during infancy and are transfusion-dependent for survival. Life-threatening which inhibits physical and metal development causing retardation. Also called Cooley’s anemia.

thalassemia major (beta)

thalassemia major (beta) hallmark s/sx

Expansion of bone marrow cavities Frontal and maxillary bossing Growth retardation Hemosiderosis - Excessive iron deposit displacement in the skin Splenomegaly

thalassemia major (beta) mgmt

Iron chelation therapy Splenectomy

for thalassemia major (beta): An iron-chelating agent has a high affinity for iron, which aids in excretion via urine or feces. Administered via continuous infusion pump over 8-12 hours, or with blood transfusions Common side effects are ocular and auditory toxicity, and growth retardation in children.

deferoxamine mesylate

Most common human enzyme deficiency with asymptomatic presentation. Affects RBCs making it vulnerable to damage or premature destruction upon exposure to triggers. Mostly associated with X-linked (high prevalence in males) but can also occur manifest in females.

Glucose-6-Phosphate Dehydrogenase Deficiency (G6PD)

G6PD: Peripheral blood smear will show ___ (degraded HgB)

Heinz bodies

s/sx of G6PD

anemia hemolysis - jaundice, abd pain, spleno/hepatomegaly systemic - fever, malaise, NV neonatal - severe jaundice

G6PD mgmt;

avoid antibacterials, antimalarial, mythelene blue, furosemide, nsaids

Refers to an increased volume of RBCs (increase hematocrit) One of the compensatory mechanism of chronic hypoxia. Impaired blood circulation occurs d/t hyperviscosity and hypervolemia

polycythemia

polycythemia vera: Characterized by hypercellularity of the bone marrow. Headache, dizziness, vertigo Blurred vision, diplopia, tinnitus Fatigue and weakness Pruritus Weight loss, night sweats Low-grade fever Gouty arthritis Ruddy complexion (Plethora) – hallmark sign

proliferative

polycythemia vera: Progression of the disease where the bone marrow gradually undergoes fibrosis (scarring), leading to a decline in its ability to produce blood cells effectively. Extramedullary hematopoiesis (blood cell production in organs like the spleen and liver) becomes more prominent to compensate. Progressive anemia Severe left upper quadrant pain and fullness Portal hypertension Severe form of s/sx from proliferative phase

splenic

polycythemia vera: Least common but most serious potential progression of PV, where the disease transforms into acute myeloid leukemia (AML) Severe bone marrow failure Rapidly worsening anemia Severe thrombocytopenia, neutropenia Progressive organomegaly (spleen or liver) Pronounced weight loss, fever, and night sweats

spent

polycythemia vera mgmt;

JAK2 gene test Phlebotomy (500mL once or twice/week) Myelosuppressive agents (hydroxyurea) Ruxolitinib (Jakafi) Radiation therapy

Hereditary coagulation defect usually transmitted to affected male by female carrier through coitus. X-linked recessive gene resulting in prolonged clotting time Found predominantly, but not exclusive, in male offspring Bleeding occurs d/t impaired ability to form fibrin clots

hemophilia

types of hemophillia: Class form d/t deficiency of factor VIII Mild: rarely develops bleeding Severe: spontaneous bleeding (hemarthroses & hematomas) frequently occurs

hemophilia A

types of hemophilia: Also known as Christmas disease d/t the deficiency of factor IX

hemophilia B

hemophilia hallmark s/sx

Hemarthrosis – hallmark Hematoma & ecchymosis Prolonged bleeding Epistaxis Spontaneous GI bleeding & hematuria Neurologic Pain, anesthesia, and paralysis (nerve compression)

hemophilia mgmt:

stop bleeding RICE Antihemophilic factor replacement Desmopressin (DDVAP) Antifibrinolytic (Tranexamic acid)

A life-threatening physiologic response with an acquired thrombotic and hemorrhagic syndrome. Not a disease but a sign of an underlying condition. Characterized by abnormal activation of clotting cascade and accelerated fibrinolysis which results to widespread clotting in small vessels, with consumption of clotting factors and platelets, so that bleeding and thrombosis occur simultaneously. “Clotting and bleeding at the same time”

Disseminated Intravascular Coagulation

Disseminated Intravascular Coagulation hallmark s/sx;

Uncontrolled internal bleeding and procedural sites low platelet prolonged PT, APTT decreased fibrinogen elevated d-dimer

Disseminated Intravascular Coagulation mgmt:

Broad spectrum antibiotics Oxygenation & fluid resuscitation, electrolyte correction Heparin IV, Vitamin K Antithrombin III (Desidurin/Atenativ)

HEMATOLOGIC DISORDERS Flashcards

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