Blood and Lymphatic Disorders

Question 1

Clinical manifestations of anemia?

Answer 1

Symptoms: fatigue, headaches, syncope, anorexia

Signs: pallor, edema

Question 2

Autoimmunity Anemias (etiologies and treatment)

Answer 2

ERYTHROBLASTOSIS FETALIS
Etiology:
1) Rh+ fetal blood breaches circulation of Rh- mother -> maternal antibodies produced -> hemolysis of fetal blood and kernicterus

Treatment:
1) RHo immune globulins before pregnancy, intrauterine transfusions, early pregnancy

AUTOIMMUNE HEMOLYTIC ANEMIA
Etiology:
1) genetic factors, infections, certain drugs -> autoantibody production against RBCs

Treatment:
1) corticosteroids, immunosuppressive drugs, RBC transfusion

Question 3

Hemoglobinopathy Anemias (etiologies and treatment)

Answer 3

SICKLE CELL ANEMIA
Etiology:
1) autosomal recessive inheritance -> hemoglobin amino acid substitution -> hemoglobin cross-binding in RBCs -> hemolysis

Treatment:
1) (symptomatic) analgesics

THALASSEMIA
Etiology:
1) genetic factors -> hemoglobin protein defects

Treatment:
1) blood transfusions, stem cell transplant

Question 4

Megaloblastic Anemias (etiologies and treatment?)

Answer 4

PERNICIOUS ANEMIA
Etiology:
1) autoantibodies produced against parietal cells and intrinsic factor -> chronic gastritis -> reduced vitamin B12 absorption -> impaired erythrocyte differentiation

Treatment:
1) vitamin B12 supplementation

FOLIC ACID DEFICIENCY:
Etiology:
1) dietary folic acid deficit -> impaired erythrocyte differentiation

Treatment:
1) vitamin B9 (folic acid) supplementation

Treatment:

Question 5

Iron Deficiency Anemia

Answer 5

Etiology:
1) dietary iron deficit -> dysfunctional hemoglobin (no O2 binding)

Treatment:
1) iron supplementation

Question 6

Polycythemia Vera

Answer 6

ETIOLOGY:

1) Absolute: idiopathic factors or hypoxemia -> increased BM hematopoiesis
2) Relative: dehydration, electrolyte imbalances -> reduced plasma volume

S/S:

1) fatigue, lightheadedness, headaches, irritability
2) thrombus, increased blood viscosity

TREATMENT:

1) treat causative factors
2) phlebotomy and/or myelosuppressive therapy

Question 7

Lead Poisoning

Answer 7

ETIOLOGY:
1) lead products inhaled or ingested -> interferes with metabolic processes (including hemoglobin production)

S/S:
1) anemia, nausea, headaches, irritability, possible cerebral edema

TREATMENT:
1) remove source of lead, chelating agents

Question 8

Agranulocytosis (Neutropenia)

Answer 8

ETIOLOGY:
1) certain drugs, poor nutrition -> destruction of neutrophils -> increased susceptibility to bacterial infection

S/S:
1) fatigue, weakness, fever, chills, rapid and weak pulse

TREATMENT:
1) cease causative factors, aggressive antimicrobial therapy

Question 9

Immune Neutropenia

Answer 9

ETIOLOGY:
1) other immune disorders, infections, certain drugs -> increase of neutrophils, but greater increase of antineutrophil antibodies -> neutrophil destruction

S/S:
1) recurrent infections, fever, fatigue, dyspnea, chills, rapid and weak pulse

TREATMENT:

1) antibiotic therapy
2) corticosteroids, G-CSF

BONUS FACTS:
1) spontaneous resolution common in infants

Question 10

Acute Lymphocytic Leukemia (ALL)

Answer 10

ETIOLOGY:
1) chromosomal abnormalities or carcinogens (UV/ionizing radiaiton, tobacco, certain viruses, certain chemicals) -> uncontrolled proliferation of immature T or B lymphocytes in BM

S/S:

1) fatigue, fever, night sweats, bone pain, weight loss, lymphadenopathy, splenomegaly
2) recurrent infections, epistaxis, menorrhagia
3) (CNS involvement) numbness, tingling, headaches

TREATMENT:
1) aggressive chemotherapy, possible hematopoietic stem cell transplant (HSCT)

BONUS FACTS:
1) 75-85% 5 year-survival-rate, prognosis much better in children

Question 11

Chronic Lymphocytic Leukemia (CLL)

Answer 11

ETIOLOGY:
1) age-related factors -> uncontrolled proliferation of mature T or B lymphocytes in bone marrow

S/S:

1) initially asymptomatic, fatigue, fever, night sweats, bone pain, weight loss, lymphadenopathy, splenomegaly, hepatomegaly
2) recurrent infections, epistaxis, menorrhagia

TREATMENT:
1) (only until patient symptomatic) chemotherapy, radiation therapy, or monoclonal antibody therapy

BONUS FACTS:
1) overall 5-year survival rate 73%

Question 12

Acute Myelogenous Leukemia (AML)

Answer 12

ETIOLOGY:
1) iatrogenic radiation or chemotherapy, tobacco, or benzene -> uncontrolled proliferation of immature (Auer rod) myeloblasts

S/S:

1) fever, fatigue, night sweats, chills, weight loss
2) recurrent infections, epistaxis, menorrhagia
3) Auer rods present during blood smear

TREATMENT:

1) chemotherapy and hematopoietic stem cell transplant
2) all-trans retinoic acid (tretinoin) if acute promyelocytic

BONUS FACTS:

1) acute promyelocytic caused by translocation error that creates PML/RAR-a gene
2) most common adult leukemia, poor prognosis in adults

Question 13

Chronic Myelogenous Leukemia (CML)

Answer 13

ETIOLOGY:
1) translocation between c9 and c22 (Philadelphia chromosome) -> uncontrolled proliferation of mature neutrophils

S/S:

1) Chronic Phase: asymptomatic, but leukocytosis present
2) Accelerated Phase: fever. fatigue, night sweats, weight loss, pain
3) Blast Crisis: anemia, blood clotting disorders, blasts 30% of BM

TREATMENT:
1) HSCT

Question 14

Lymphedema

Answer 14

ETIOLOGY:
1) hypersensitivity reactions (inflammation), infections, neoplasms -> mechanical obstruction of lymphatic vessels

S/S:
1) painless swelling in extremities, possible limited ROM

TREATMENT:

1) antibiotics if infectious
2) elevate affected limbs above heart level, compression stockings, diuretics, surgery

Question 15

Lymphangitis

Answer 15

ETIOLOGY:
1) break in skin -> bacteria invade lymphatic vessels

S/S:
1) tenderness, swelling, cellulitis, fever, fatigue, malaise,

TREATMENT:
1) antibiotics and surgical drainage

Question 16

Hodgkin’s Lymphoma

Answer 16

ETIOLOGY:
1) family history, ionizing radiation, chemotherapy, or Epstein-Barr virus (EBV) -> uncontrolled prolifertion of immature (Reed-Sternberg) lymphocytes in lymph nodes

S/S:
1) lymphadenopathy, pruritis, fatigue, fever, night sweats, weight loss

TREATMENT:
1) ABVD combined chemotherapy, HSCT

BONUS FACTS:
1) 90% 5-year survival rate

Question 17

Non-Hodgkin’s Lymphoma

Answer 17

ETIOLOGY:
1) family history, EBV/HCV/HIV, prior radiation or chemotherapy -> uncontrolled proliferation of lymphocytes in lymph nodes

S/S:
1) lymphadenopathy, enlarged tonsils or adenoid gland, bone pain, GI symptoms, fatigue, B symptoms (fever, weight loss, night sweats)

TREATMENT:
1) CHOP combined chemotherapy, local radiation therapy, and/or HSCT

BONUS FACTS:

1) classified into: indolent (40%), aggressive (50%), and highly aggressive (5%)
2) 69% 5-year survival rate

Question 18

Transfusion Incompatibility Reaction

Answer 18

ETIOLOGY:
1) incompatible (ABO- or Rh) blood enters circulation -> histamine and serotonin released -> agglutination, hemolysis, possible DIC

S/S:

1) fever, chills, nausea, dyspnea, hives
2) risk of renal failure

TREATMENT:

1) observe vitals every 15 minutes during any transfusions
2) antihistamines

Question 19

Hemophilia

Answer 19

ETIOLOGY:
1) X linked recessive -> defective clotting factor VIII -> impaired hemostasis

S/S:
1) prolonged bleeding, epistaxis, ecchymosis, joint pain or swelling

TREATMENT:
1) avoid bleeding situations, clotting factor VII administration, possible blood transfusions

BONUS FACTS:
1) Von Willebrand Disease affects von Willebrand Factor (vWF), which is responsible for platelet adhesion

Question 20

Idiopathic Thrombocytopenia Purpura (ITP)

Answer 20

ETIOLOGY:
1) autoantibodies produced against platelets -> impaired coagulation

S/S:
1) epistaxis, prolonged bleeding, petechia, ecchymosis, menorrhagia, GI bleeding

TREATMENT:
1) corticosteroids, IV immune globulin, splenectomy

BONUS FACTS:
1) spontaneous resolution common in children

Question 21

Disseminated Intravascular Coagulation (DIC)

Answer 21

ETIOLOGY:

1) certain cancers, pregnancy, sepsis, endothelial damage -> thrombin active in systemic circulation -> fibrin and platelets exhausted
* presence of thrombin -> fibrinolytic system activation -> bleeding

S/S:

1) weakness, tachycardia, air hunger
2) purpura, petechia, hematoma, hematemesis, hematuria, bloody stools

TREATMENT:

1) treat causative factors
2) IV heparin to prevent clotting, platelet and cF replacement