Usmle Step 1 Fa Heme/onc-Rbc And Rbc Disordes

Front 1

Difference b/w anti AB antibodies and anti Rh antibodies?

Back 1

Rh antibodies are IgG (cross placenta)
AB antibodies are IgM (do not cross placenta)

Front 2

Macrovalocytes seen in?

Back 2

Megaloblastic anemia (also hypersegmented PMN's), and marrow failure

Front 3

Helmet cells, schistocytes seen in?

Back 3

DIC, TTP/HUS, traumatic hemolysis

Front 4

Bite cell seen in?

Back 4

G6PD deficiency

Front 5

Teardrop cell seen in?

Back 5

Myeloid metaplasia w/myelofibrosis

Front 6

Acanthocytes (spur cells) seen in?

Back 6

Spiny appearance in liver disease and abetalipoproteinemia

Front 7

Target cell seen in?

Back 7

HbC disease
Asplenia
Liver disease
Thalassemia

Front 8

Burr cells seen in?

Back 8

TTP/HUS

Front 9

Basophilic stippling seen in?

Back 9

Thalassemias
Anemias of chronic disease
Iron deficiency
Lead poisoning

Front 10

Heinz bodies
(a) etiology
(b) seen in?

Back 10

(a) oxidation of iron from ferrous to ferric leads to denatured Hgb precipitation and damage to RBC membrane; forms bite cells
(b) alpha thalassemia, G6PD deficiency

Front 11

Howell Jolly bodies
(a) etiology
(b) seen in?

Back 11

(a) basophilic nuclear remnants found in RBC's
(b) hypo/asplenic patients

Front 12

Etiologies of microcytic, hypochromic anemias (MCV<80)

Back 12

Iron deficiency
Thalassemias
Lead poisoning
Some sideroblastic anemias

Front 13

Differential feature b/w iron deficiency and thalassemia hypochromic, microcytic anemias

Back 13

In thallassemia you have increased # of microcytes
IN iron deficiency you have decreased # of microcytes

Front 14

Etiologies of macrocytic anemias (MCV>100)

Back 14

Megabloblastic (vitB12/folate deficiency)
Drugs that block DNA synthesis (sulfa drugs, phenytoin, AZT)

Front 15

Decr serum haptoglobin
Incr serum LDH indicate?

Back 15

RBC hemolysis

Front 16

Abdominal pain
Pink urine
Polyneuropathy
Psychological disturbances
Precipitated by drugs

Back 16

Porphyria

Front 17

Lead poison effect on heme synthesis
(a) enzyme(s) inhibited
(b) accumulated substrate in urine

Back 17

(a) ferrocheletase; ALA dehydratase
(b) coproporphyrin & ALA

Front 18

Acute intermittent porphyria
(a) enzyme inhibited
(b) accumulated substrate in urine

Back 18

(a) uroporphyrinogen I sunthase
(b) prophobilinogen and gamma ALA

Front 19

Porphyria cutanea tarda
(a) enzyme inhibited
(b) accumulated substrate in urine

Back 19

(a) uroporphyrinogen decarboxylase
(b) uroporphyrin

Front 20

Acute intermittent porphyria presentation (inheritence)

Back 20

Autosomal Dominant
Episodic expression
Abd pain
anxiety/confu/paranoia
Muscle weakness
Port wine urine in some patients
NO photosensitivity

Front 21

Porphyria cutanea Tarda presentation

Back 21

Photosensitivity
Skin inflammation and blistering
Cirrhosis often associated

Front 22

Lead poisoning presentation

Back 22

Microcytic sideroblastic anemia
Basophilic stippling
HA, nausea, memory loss, abd pain, diarrhea
Lead lines in gums
Neuropathy (claw hand, wrist drop)

Front 23

Ferritin

Back 23

Primary iron storage protein of body

Front 24

Transferrin

Back 24

Transport iron in blood

Front 25

Haptoglobin

Back 25

Binds free heme in blood

Front 26

Iron deficiency
(a) serum iron
(b) transferrin/RIBC (indirectly measures transferrin)
(c) ferritin
(d) % transferrin sat (serum Fe/TIBC)

Back 26

(a) serum iron: low (primary)
(b) transferrin/TIBC (indirectly measures transferrin): incr
(c) ferritin: low
(d) % transferrin sat (serum Fe/TIBC): very low

Front 27

Chronic disease
(a) serum iron
(b) transferrin/TIBC (indirectly measures transferrin)
(c) ferritin
(d) % transferrin sat (serum Fe/TIBC)

Back 27

(a) serum iron: low
(b) transferrin/TIBC (indirectly measures transferrin): low
(c) ferritin: high (primarily)
(d) % transferrin sat (serum Fe/TIBC): nml

Front 28

Hemochromatosis
(a) serum iron
(b) transferrin/TIBC (indirectly measures transferrin)
(c) ferritin
(d) % transferrin sat (serum Fe/TIBC)

Back 28

(a) serum iron: high (primary)
(b) transferrin/TIBC (indirectly measures transferrin): low
(c) ferritin: high
(d) % transferrin sat (serum Fe/TIBC): very high

Front 29

Pregnancy/OCP use
(a) serum iron
(b) transferrin/TIBC (indirectly measures transferrin)
(c) ferritin
(d) % transferrin sat (serum Fe/TIBC)

Back 29

(a) serum iron: nml
(b) transferrin/TIBC (indirectly measures transferrin): high (primary)
(c) ferritin: nml
(d) % transferrin sat (serum Fe/TIBC): low

Front 30

Aplastic anemia

Back 30

Pancytopenia
Due to failure or destruction of multipotent myeloid stem cells

Front 31

Etiologies of aplastic anemia

Back 31

Radiation
Benzene
Chloramephnicol
Alkylating agents
Antimetabolites
Viruses (B19, EBV, HIV)
Fanconie's anemia (inherited defect in DNA repair)
Idiopathic
May follow acute heptatitis

Front 32

Symptoms of aplastic anemia

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Fatigue
Malaise
Pallor
Purpura
Mucosal bleeding
Petechiae
Infection

Front 33

Pathologic features of aplastic anemia

Back 33

Pancytopenia w/normal cell morphology, hypocellular bone marrow w/fatty infiltration. Diagnose with bone marrow biopsy

Front 34

Treatment of aplastic anemia

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Withdrawal of offending agent.
Immunesuppressive regiments
Allogeneic BMT
RBC and platelet transfusion
GCSF, GM CSF

Front 35

Myelophthisic anemia

Back 35

Displacement of hematopoietic bone marrow by infiltrating tumor

Front 36

Mutation in sickle cell anemia

Back 36

Valine fro glutaminc acid in position 6 of beta chain of Hgb

Front 37

Sickel trait manifestations

Back 37

40% have a sickle crisis in extreme conditions (low oxygen or dehydration)

Front 38

Sickle prep

Back 38

Blood sample treated with a reducing agent (sickled cells may be seen)

Front 39

Diagnosis of sickle cell anemia

Back 39

Hgb electrophoresis

Front 40

Advantage of heterozygotes (sickle trait)

Back 40

Protective against falciparum malaria

Front 41

Complications of sickle cell anemia patients

Back 41

Aplastic crisis (parvovirus B19)
Autosplenectomy
Incr risk of encapsulated organism
Salmonella osteomyelitis
Painful crisis
Renal papillary necrosis
Splenic sequestration

Front 42

Therapies for sickle cell anemia

Back 42

Hydroxyurea (incr HgbF)
Bone marrow transplant

Front 43

"crew cut" skull on x ray seen in? why?

Back 43

Sickle cell anemia and thallasemia
Due to marrow expansion from incr erythropoiesis

Front 44

HbC disease

Back 44

Different beta chain mutation; patients w/HbC or HbSC have a milder disease than HbSS patients

Front 45

Defect in alpha thalassemia

Back 45

Defect in 1 to 4 alpha globin genes
Alpha globin is underproduced w/nocompensatory incr in any of the other chains

Front 46

1 deleted alpha globin chain mutation

Back 46

Silent carrier

Front 47

2 deleted alpha globin chain mutation

Back 47

Alpha thal trait

Front 48

3 deleted alpha globin chain mutation

Back 48

HbH disease; hemolysis and anemia

Front 49

4 deleted alpha globin chain mutation

Back 49

Hydrops fetalis (bart Hb); intrauterine death

Front 50

Alpha thalassemia is prevalent where geographically?

Back 50

Asia and africa

Front 51

Defect and result in beta thalassemia

Back 51

Mutations in splicing sites and promoter sequences for beta globin gene
Excess of alpha chains causes Hgb aggregates (insoluble)
Intra and extramedullary hemolysis
Extramedullary hematopoiesis

Front 52

HbS/beta thal

Back 52

Mild to moderate sickle disease dependeing on amnt of beta globin production

Front 53

Treatment of beta thal major

Back 53

Severe anemia requiring blood transfussions

Front 54

Complications of beta thal major

Back 54

Heart failure due to secondary hemochromatosis

Front 55

Hemolytic anemia: hemoglobinuria indicates?

Back 55

Intravascular hemolysis

Front 56

Hemolytic anemia: jaundice indicates?

Back 56

Extravascular hemolysis

Front 57

Warm agglutinin hemolytic anemia
(a) mechanism
(b) etiologies

Back 57

(a) IgG autoimmune hemolytic anemia; accelerated RBC destruction in liver Kupffer cells and spleen)
(b) SLE, CLL, certain drugs (alpha methyl dopa)

Front 58

Cold agglutinin hemolytic anemia
(a) mechanism
(b) etiologies

Back 58

(a) acute anemia triggered by cold; IgM antibodies against RBC
(b) mycoplasma, mononucleosis, idiopathic, lymphoma

Front 59

Hereditary spherocytosis defect

Back 59

Ankyrin, band 3.1 or spectrin defectl; cause RBCs to be less pliable and vulnerable to destruction in the spleen

Front 60

MCHC and RDW in hereditary spherocytosis

Back 60

Increased

Front 61

Symptoms of hereditary spherocytosis

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Anemia, jaundice, splenomegaly, cholelithiasis

Front 62

Treatment of hereditary spherocytosis

Back 62

Splenectomy

Front 63

Presplenectomy smear for hereditary spherocytosis

Back 63

Cells lacking central pallor; reticulocytosis

Front 64

Postsplenectomy smear

Back 64

More spherocytes and Howel Jowel bodies

Front 65

Paroxysmal nocturnal hemoglobinuria cause (defect)

Back 65

Intravascular hemolysis due to memraben defect; incr sensitivity of RBC to lytic activity of complemement (impaired synth of GPI anchor/decay accelerating factor in RBC membrane).
See hemosiderin in urine

Front 66

G6PD deficiency defect (inheritence)

Back 66

XL defect of hexose monophsophate shunt enzyme
Decreased regeneration of NADPH (and thus glutathione)
Older cells unable to tolerate oxidative stress

Front 67

Cause of G6PD related anemia

Back 67

Drugs (sulfa, quinine, nitrofurantoin)
Infections (particularly viral)
Certain foods (fava beans)

Front 68

Smear in G6PD deficiency

Back 68

Reticulocytosis and Heinz bodies (Hgb degradation products)