Hematology/Oncology

Question 1

Thalassemia

Answer 1

Eti: Hereditary disorders characterized by reduction in the synthesis of globulin chains.
Minor: no significant clinical impact (insignificant microcytic anemia)
Alpha major: no hemoglobin produced: stillborn fetus.
Beta major: stunted growth, bony deformities, HSM, jaundice, cirrhosis, thrombophilia.
Alpha: predominantly asian descent
Beta: mediterranean descent

Question 2

Folic acid deficiency (vit B9)

Answer 2

Eti: most common cause adequate dietary intake (ETOH and anorectic patients
S/sx: similar to B12 def, but none of the neurologic def.

Question 3

B12 deficiency

Answer 3

Eti: Obtained by diet. Pernicious anemia: auto-antibodies
S/sx: Glossitis, GI disturbances (anorexia and diarrhea)…..
Prevention: nutrition if deficient
Treatment: parenteral b12 if severe

Question 4

Macrocytic anemias

Answer 4

Most macrocytic anemias are megaloblastic.
Megaloblastic: enlarged RBC precursor with non-condensed chromatin.
Megaloblastic type: usually B12 or folate deficieny
Nonmegaloblastic: No DNA synthesis impairment. Disorders with increased membrane surface area, accelerated erythropoiesis, ETOH, COPD

Question 5

Aplastic anemia

Answer 5

Bone marrow failure characterized by peripheral pancytopenia and marrow hypoplasia.
Usually normocytic anemia.

Question 6

Anemia due to liver disease

Answer 6

Macrocytic, but no megaloblastic
Think about clotting issues, increased bleeding (GI bleeds).
Look for ascites, pancytopenia, caput medusae, (thinking about portal hypertension).
Diag: Macrocytic RBCs, with target cells

Question 7

Anemia due to kidney disease

Answer 7

Patho: kidneys make majority of EPO, liver makes a little
S/s: fatigue, dyspnea, syncope, confusion, weakness
Diag: macrocytic
Tx: exogenous EPO

Question 8

Anemia of chronic disease

Answer 8

Patho: anemia of inflammation (IBD, RA, CA are examples).
Generally normocytic
Tx: treat the disease

Question 9

Sickle cell anemia

Answer 9

Patho: Abnormal hemoglobin causes sickling
S/sx: chronic hemolytic anemia: jaundice, gallstones, splenomegaly, vasoocculsion
Hemolytic crisis: Spleen sequesters sickle cells
Aplastic crisis: bone marrow compensation is reduced by infection or folate def.
Labs: low crit, reticulocytosis, howell-jolly bodies
Tx: folic acid, hydroxyurea (makes rbc’s more flexible)

Question 10

Paroxysmal nocturnal hemoglobinuria

Answer 10

hematopoietic stem cell disorder: RBC membrane sensitivity to complement, leading to lysis
S/sx: First morning urine is reddish brown
Triad: hemolysis, pancytopenia, thrombosis

Question 11

Sickle cell trait

Answer 11

Sickling happens at low O2 concentrations

Question 12

Hereditary spherocytosis

Answer 12

decrease surface-to-volume ratio and a spherical RBC shape. Get trapped in spleen.
S/sx: Jaundice, cholecystitis, splenomegaly

Question 13

G6PD deficiency

Answer 13

Patho: Hemolytic anemia from RBCs not able to deal with oxidative stresses. Hemoglobin denatures and forms heinz bodies.
S/sx: Hemollysis at time of infection or exposure to certain drugs. Jaundice, pale skin, back/belly pain.
Lab: Smear shows: bite cells, blister cells, heinz bodies

Question 14

Autoimmune hemolytic anemia

Answer 14

Eti: acuired IgG autoantibody binds to RBCs.
Examples: SLE (10% of these patients), CLL, lymphoma

Question 15

Hemochromatosis

Answer 15

Dysregulation of iron absorption: in the liver, pancreas, heart, adrenals, testes, pituitary and kidneys
S/sx: most asymp,
Advanced D: hepatomegaly, splenomegaly, increased pigmentation, spider angiomas

Question 16

Acute Lymphoid Lekuemia (ALL)

Answer 16

Most common acute leukemia in children
Epi: 5-6yo or around 50
S/sx: hepatosplenomegaly, LAD, bone pain, anemia, thrombocytopenia, petechiae
Labs: CBC, manuel diff, …

Question 17

Acute Myeloid Leukemia (AML)

Answer 17

Epi: median age presentation: 60
Patho: neoplasm derived from hematopoieti precursors that ifive rise to granulocytes, monocytes, erythrocytes, platelets.
S/sx: Malaise, fatigue, dyspnea, wt. loss, gingival hyperplasia…
Labs: abnormal CBC: high or low WBC, AUER RODs,

Question 18

Chronic lymphocytic leukemia (CLL)

Answer 18

Neoplasm of more mature B cells.
Expansion of a neoplastic clone results in an increased # of small long-lived lymphocutes that are immunocompetent. Results in immunosuprresion
Epi: Most common leukemia in US, 2x men compared to women
Labs: lymphocytosis >5000/ mL, 75-98 % of circulating cells are lymphocytes, SMUDGE cells
Tx: no cure

Question 19

Chronic Myeloid Luekemia (CML)

Answer 19

Eti: over production of myeloid cells, but are not as effective as regular myeloid cells
Epi: median age presentation is 50.
S/sx: fatigue, night sweats, low grade fever, abdominal fullness,
Pathophys: Philadelphia 22 chromosome
Labs: Elevated WBC count:
Tx: Imatinib (targeted therapy)

Question 20

Polycythemia vera

Answer 20

An acquired myeloproliferative disorder causing overproduction of 3 hematopoietic cell lines, but most elevated are the RBCs
S/sx: HA, dizziness, tinnitus, blurred vision, fatigue, generalized pruritus
Phys exam: engorged retinal veins, spleen is palpable in 75% of cases.
Dx: HCT >54% men, >51% in women
Complications: Thrombosis, increased viscosity of blood and abnormal platelet function.

Question 21

Hodgkin’s disease

Answer 21

Eti: Malignant cell is a B lymphocyte
Epi: bimodal, 20-30yo and >50yo
Cell type: Reed-Sternberg cells
S/sx: painless lymphadenopathy, constitutional symptoms (fever, wt. loss, night sweats, generalized pruritis)
Distribution: contiguous lymph node spread.

Question 22

Non-hodgkin’s lymphoma

Answer 22

Heterogenous group of cancers of lymphocytes usually presenting as enlarged lymph nodes.
S/sx: Painless lymphadenopathy, consititional sxs (fever, night sweats, wt loss).
Dx: lymph node biopsy
Distribution: noncontiguous lymph node spread

Question 23

Multiple myeloma

Answer 23

Eti: malignancy of plasma cells. Replacement of bone marrow, bone destruction, and paraprotein formation.
Abnormal proteins can cause kidney problems.
Epi: mean age 65 years.
Patho: Paraprotein: abnormal immunoglobulin that is produced by abnormal proliferation of plasma cells.
S/sx: Bone pain (often spine, hips, ribs, proximal long bones). Infection, Kidney failure.
Labs: Anemia, hypercalcemia, proteinuria, elevated ESR, BENCE JONES PROTEINS.

Question 24

Burkitt’s Lymphoma

Answer 24

Very fast growing non-hodgkins lymphoma
Epi: more men, mean age 30
S/sx: 65% present with abdominal mass. Lymphadenopathy. FEVER, NIGHT SWEATS,