Blood and Lymphatic Disorders

Question 1

Microcytic, developing countries ( reduced intake), developed countries (blood loss, G.I. or menstruation), pica, fingernail spooning

Answer 1

Iron deficiency anemia

Question 2

Microcytic, common among hospitalized, chronic/systemic inflammation suppresses erythropoiesis

Answer 2

Anemia of chronic disease

Question 3

Microcytic, unstable RBCs cause hemolysis, β-thalassemia is much more severe, skeletal deformation, hair-on-end skull

Answer 3

Thalassemia (α and β)

Question 4

Microcytic, interferences with enzymes for heme synthesis, which impairs the ability to incorporate iron into heme, basophilic stippling

Answer 4

Lead poisoning

Question 5

Macrocytic (megaloblastic), associated with gastric atrophy or malabsorption, elderly, fatigue, weakness, neuropathies (distinguish from folate deficiency anemia

Answer 5

Vitamin B12 deficiency anemia (pernicious anemia)

Question 6

Macrocytic (megaloblastic), poor diet (processed), fatigue, weakness, sore tongue

Answer 6

Folate deficiency anemia

Question 7

Normocytic, hemorrhage, burns, G.I. bleed, bloody diarrhea

Answer 7

Acute blood loss

Question 8

Normocytic, RBC membrane mutations, causes spherical RBCs, cells look dark on peripheral smear, splenectomy is common treatment

Answer 8

Hereditary spherocytosis

Question 9

Normocytic, myeloid stem cells are suppressed, T cells attack marrow, failure to produce RBCs, granulocytes, platelets, commonly idiopathic, splenomegaly is characteristically absent, petechiae (loss of platelets)

Answer 9

Aplastic anemia

Question 10

Normocytic, RBCs are sensitive to complement-mediated hemolysis, sleeping causes reduced pH, complement is slightly activated, wake with hemoglobinuria in morning (paroxysmal)

Answer 10

Paroxysmal nocturnal hemoglobinuria

Question 11

Normocytic, β-globin mutation, microvascular thrombosis (crisis), acute chest syndrome, stroke, African Americans, stunted growth

Answer 11

Sickle cell anemia

Question 12

X-linked disorder of deficient G6PD, failure to protect RBCs against oxidation (infection, NSAIDs), Heinz bodies, bite cells, 3 day lag period

Answer 12

Glucose-6-phosphate dehydrogenase (G6PD) deficiency

Question 13

Warm antibody: active around body temperature, IgG opsonization. Cold antibody: active around 85 degrees F, IgM opsonization. Drug induced

Answer 13

Immune hemolytic anemia

Question 14

Plasmodium falciparum (Anopheles mosquito), Africa and SE Asia, lethal, intravascular hemolysis, merozoite showers, fever, splenomegaly, may cause CNS or renal damage

Answer 14

Malaria

Question 15

increased RBC mass

1. Relative: dehydration
2. Absolute: reaction to elevated erythropoietin (hypoxia)

Answer 15

Polycythemia

Question 16

increased RBC mass due to autonomous production, normal erythropoietin, chronic myeloproliferative disorder, hypercellular marrow, overexpression of myeloid stem cell, JAK2 gene mutation, hepatosplenomegaly, thrombosis, headache, vertigo, stroke, pruritus after bathing

Answer 16

Polycythemia vera

Question 17

WBC disorder, Infectious mononucleosis

Answer 17

Epstein-Barr virus (EBV), fever, pharyngitis, lymphadenitis, Monospot test for atypical lymphocytes

Question 18

WBC disorder, Bartonella henselae, most common among pediatrics

Answer 18

Cat-scratch disease

Question 19

B cells, predictable metastasis, Reed-Sternberg cells, painless lymphadenopathy, night sweats, cachexia, anemia, pruritis, history of EBV infection or Agent Orange exposure

Answer 19

Hodgkin lymphoma

Question 20

Non-hodkin, aggressive, rapid onset, T or B cells, children

Answer 20

Acute lymphoblastic leukemia

Question 21

Non-hodkin, B cells, insidious, most common leukemia of adulthood, pancytopenia, infections, anemia

Answer 21

Chronic lymphocytic leukemia

Question 22

Non-hodkin, B cells, variable aggressiveness, most over 50 years old, lymph nodes have a nodular (follicular) appearance

Answer 22

Follicular lymphoma

Question 23

Non-hodkin, B cells, most common lymphoma of adulthood, fatal if untreated, responds well to chemotherapy

Answer 23

Diffuse large B cell lymphoma

Question 24

Non-hodkin, B cells, t(8; 14), children, Africa, “starry sky” histology, rapid growth, facial involvement

Answer 24

Burkitt lymphoma

Question 25

plasma cell tumor, elderly, “punched out” lesions of calvaria and vertebral bodies, Bence-Jones proteins, M-spike, inverted albumin-globulin ratio (A-G ratio), invasive, incurable

Answer 25

Multiple myeloma

Question 26

anaplastic myeloid cells, aggressive, replace marrow, suppress hematopoiesis (marrow failure), bleeding, bone pain, pallor, cachexia, poor prognosis

Answer 26

Acute myelogenous leukemia

Question 27

disordered hematopoieses, pancytopenia, many transform into acute myelogenous leukemia, poor prognosis

Answer 27

Myelodysplastic syndromes (pre-leukemia)

Question 28

hyperplastic myeloid cells produce too many RBCs, WBCs, and platelets, eventual “spent phase,” may transform into acute myelogenous leukemia

Answer 28

Chronic myeloproliferative disorders

Question 29

Philadelphia chromosome t(9;22)

Answer 29

Chronic myelogenous leukemia

Question 30

diffuse marrow fibrosis, extramedullary hematopoiesis, poikilocytes, diagnosed in late stages

Answer 30

Primary myelofibrosis

Question 31

Birbeck granules (tennis rackets), skin lesions, lymphadenopathy

Answer 31

Langerhans cell histiocytes

Question 32

massive tissue injury (birthing, sepsis, malignancy, severe trauma), widespread microvascular clotting (stroke, M.I.); may consume all clotting factors (widespread hemorrhage), highly lethal

Answer 32

Disseminated intravascular coagulation

Question 33

abnormal Willebrand factor (vWF) complex, bleeding gums, underdiagnosed (1% of U.S. population)

Answer 33

von Willebrand disease

Question 34

mutation of coagulation factor VIII, X-linked recessive

Answer 34

Hemophilia A

Question 35

mutated coagulation factor IX mutation, X-linked recessive, Christmas disease

Answer 35

Hemophilia B

Question 36

increase permeability of the glomeruli causing massive proteinuria, hypoalbuminemia in plasma (generalized edema); many causes: diabetes, lupus, Sjögren’s syndrome, sepsis, syphilis, HIV, amyloidosis, vasculitis, cancer, adverse drug reactions

Answer 36

Nephrotic syndrome

Question 37

most commonly from a bacterial urinary tract infection (upper urinary tract infection) from E. coli, pyrexia, dysuria, flank pain

Answer 37

Pyelonephritis (inflammation of the renal pelvis)

Question 38

genetic disease that results in massive cysts to develop within the kidneys, children = autosomal recessive, adults = autosomal dominant, common, cause of 10% of chronic renal failure

Answer 38

Polycystic kidney disease

Question 39

most common cancer of the kidney among adults

Answer 39

Renal cell carcinoma

Question 40

common childhood cancer, 2-5 years old

Answer 40

Wilms tumor

Question 41

general term for inflammation of the liver, which damages hepatocytes

Answer 41

Hepatitis

Question 42

types A-E, common cause of liver cirrhosis

Answer 42

Viral

Question 43

fecal-oral; 40% of hepatitis cases in U.S., never causes chronic liver disease, “A” involves contact with the arse

Answer 43

Hep. A virus (HAV):

Question 44

sexual contact, parenteral, breast feeding, birth; 45% of cases in U.S., causes 10% of chronic liver disease, “B” is from blood, birth, and “bonking” (sexual intercourse)

Answer 44

Hep. B virus (HBV)

Question 45

sexual contact, parenteral; 20% of cases in U.S., causes 80% of chronic liver disease, “C” is very chronic

Answer 45

Hep. C virus (HCV)

Question 46

sexual contact, parenteral; 1% of cases in U.S., causes 5% of chronic liver disease

Answer 46

Hep. D virus (HDV)

Question 47

fecal-oral; very rare, more common in pregnant females, never causes chronic liver disease, “vowels” (A, E) never cause chronic liver disease)

Answer 47

Hep. E virus (HEV)

Question 48

females, no serologic evidence of viral infection, high levels of autoantibodies (IgG), associated with RA, Sjögren’s syndrome, and ulcerative colitis

Answer 48

autoimmune hepatitis

Question 49

death of hepatocytes with diffuse hepatic nodular fibrosis (loss of liver function), result of viral hepatitis, fatty liver disease (below), autoimmune disease, or iron overload (Wilson disease, below); commonly causes portal hypertension, increased risk of hepatocellular carcinoma (cancer), and may cause lethal hepatic encephalopathy

Answer 49

cirrhosis

Question 50

: increased blood pressure within the portal venous system, result of cirrhosis, produces abdominal swelling (ascites), splenomegaly, or hepatic encephalopathy (below)

Answer 50

portal hypertension

Question 51

causes hepatocellular steatosis (fat accumulation)

Answer 51

Fatty liver disease (FLD)

Question 52

steatosis, alcoholic hepatitis, cirrhosis, develops after 10-15 years of chronic alcoholism

Answer 52

Alcoholic FLD

Question 53

steatohepatitis, cirrhosis; associated with metabolic syndrome, obesity, type 2 diabetes mellitus, hypertension, and dyslipidemia, becoming more common among pediatrics in the U.S. due to obesity epidemic

Answer 53

Non-alcoholic FLD

Question 54

excessive deposition of iron in the liver, pancreas, and heart; adult onset, may also develop among individuals with multiple blood transfusions (thalassemia), males are most commonly effected (women have menstruation to lose iron)

Answer 54

Hemochromatosis (iron-overload)

Question 55

accumulation of toxic levels of copper, autosomal recessive, damages the liver, brain, and eyes, childhood onset, psychosis, tremors, Kayser-Fleischer rings encircle the iris

Answer 55

Wilson disease

Question 56

liver failure results in shunting of toxins from the liver and into systemic circulation, damages the brain, cerebral edema, causes tremor (asterixis), Babinski sign, confusion, stupor, seizures, coma, or death

Answer 56

Hepatic encephalopathy

Question 57

common in U.S. (20% of adults), may be cholesterol or pigment (bilirubin)stones, most are asymptomatic, may lead to acute cholecystitis, sever epigastric/right upper quadrant pain when symptomatic, no relationship with any particular diet

Answer 57

Cholelithiasis (gallstones)

Question 58

commonly due to obstruction of the cystic duct with gallstones (cholelithiasis), pyrexia, pain in the right upper quadrant or epigastric region, commonly refers pain to the right scapula (Boas’ sign)

Answer 58

Cholecystitis (inflammation of the gallbladder)