Fire Facts

Question 1

What protein allows RBCs to change shape as they pass through vessels?

Answer 1

Spectrin

Question 2

What name is given to immature erythrocytes in circulation?

Answer 2

Reticulocytes

Question 3

What pathologic form of RBC would you see in the following diseases? Lead poisoning

Answer 3

Basophilic Stippling

Question 4

What pathologic form of RBC would you see in the following diseases? G6PD Deficiency

Answer 4

Bite cells and Heinz Bodies

Question 5

What pathologic form of RBC would you see in the following diseases? DIC or TTPHUS

Answer 5

Schistocytes - fragmented RBC

Question 6

What pathologic form of RBC would you see in the following diseases? Abetalipoproteinemia or liver disease

Answer 6

Acanthocytes (spur cells) - irregularly spiked

Question 7

What pathologic form of RBC would you see in the following diseases? Asplenia

Answer 7

Howell-Jolly bodies and Target cells

Question 8

What pathologic form of RBC would you see in the following diseases? Uremia/Renal Failure

Answer 8

Echinocytes (burr cells) - regular uniform spikes

Question 9

What pathologic form of RBC would you see in the following diseases? Rounded cell that has lost its concave shape

Answer 9

Sherocytosis - Hereditary Spherocytosis

Question 10

What pathologic form of RBC would you see in the following diseases? Liver disease

Answer 10

Schistocytes or acanthocytes

Question 11

What pathologic form of RBC would you see in the following diseases? Infarcted spleen

Answer 11

Howell-Jolly bodies

Splenectomy is Tx for: Immune thrombocytic purpura (ITP), Sickle cell anemia, or Px with spleen trauma

Question 12

Px presents with anaphylaxis on exposure to blood products with IgA.
What pathology is at hand?

Answer 12

Selective IgA deficiency

Question 13

Px presents with corse facial features, abscesses, eczema.

What pathology is at hand?

Answer 13

Hyper-IgE Syndrome (Job Syndrome)

Question 14

Px presents with thrombocytopenia, purpura, infections, eczema.
What pathology is at hand?

Answer 14

Wiskott-Aldrich syndrome

Question 15

Px presents with delayed separation of the umbilicus.

What pathology is at hand?

Answer 15

Leukocyte adhesion deficiency type I.

Phagocyte dysfunction disorder.

Question 16

Px presents with Neuro defects, partial albinism, recurrent infections.
What pathology is at hand?

Answer 16

Chédiak-Higashi Syndrome

Also a phagocyte dysfunction disorder

Question 17

What 3 hematologic changes would you expect to see in a Px without a functional spleen?

Answer 17

Thrombocytosis
Howell-Jolly bodies
Target cells

Question 18

Where does fetal erythropoiesis take place??

Answer 18

“Young liver synthesizes blood”

• Yolk Sac (3 -8 weeks)
• Liver and Spleen (migrated to liver from mesonephros after a couple of weeks)
• Bone marrow (~28wks)
• Vertebrae, ribs, and pelvis (axial skeleton in adults)

Question 19

PT describes the following symptoms:
Encephalopathy, memory loss, headaches, foot/wrist drop, abdominal colic, and renal colic (eventually has renal failure). What pathology is at hand?

Answer 19

Lead poisoning
These symptoms are the non-hematologic signs which also include:
Lead lines on bone X-ray, and on gingivae (Burton lines)

Question 20

What triggers sickling of RBCs?

Answer 20

Hypoxemia
Dehydration
Acidosis

May cause a sickle cell crisis

Question 21

Pt presents with cyanosis of the fingers and toes, with hemolytic anemia. What disease is this?

Answer 21

Cold autoimmune hemolytic anemia

—> cold involves IgM
—> Warm involves IgG

Question 22

Pt presents with hematuria in the mornings, and fragile RBCs. What disease is at hand? What is happening?

Answer 22

Paroxysmal nocturnal hemoglobinuria

RBC are undergoing premature hemolysis by way of complement, and thus thrombosis, impaired bone marrow function, and hemolytic anemia make the disease life threatening.

Question 23

Basophilic nuclear remnants in RBCs. What is this called?

Answer 23

Howell-Jolly bodies

Question 24

Pt undergoes autosplenectomy… what is the associated disease? What is the treatment of choice?

Answer 24

Sickle cell

Tx of choice for crisis: hydroxyurea

Question 25

Pt presents with lower extremity purpura, arthralgias, and renal disease, what is the most likely diagnosis?

Answer 25

Henoch-Schonlein purpura (type of vasculitis)

Question 26

What are the 3 constitutional B symptoms?

Answer 26

Low grade fever
Night sweats
Weight loss

Question 27

What is the translocation in Philadelphia chromosome? What is the specific mutation? What disease is more commonly seen with this mutation?

Answer 27

t (9;22) —> creates a mutation in bcr-abl (an oncogene) to be always active

Seen in: CML (philly CreaML cheese)

Question 28

Why is AML more common in Pts with Down syndrome?

Answer 28

The translocation at t (8;21) would give 3 copies of the translocation, thus making AML more common in these patients

Question 29

Under microscopy, plasma cells show a nucleus that is packed with a “clock face” appearance of chromatin in the nucleus. The cytoplasm just outside the nucleus is cleared out and is called the peri nuclear hof. RBC have a Rouleaux formation (stacked like coins). Lyric bone lesions like Swiss cheese on xrays, like the skull. What is the pathology at hand?

Answer 29

Multiple myeloma

Question 30

What are the 4 MC clinical features of Multiple myeloma? What urine test is helpful for diagnosis?

Answer 30

-Anemia
-Renal insufficiency
-Back pain (due to incr. osteoclast func, dec. OB function
-Hypercalcemia
(Also, monoclonal antibody spike “M-Spike” on serum protein electrophoresis
Immunoglobulin light chain in urine (no proteinuria), but you see Bence-Jones proteins by way of urine protein electrophoresis (UPEP)

Seen In an older Px usually

Question 31

How do you differentiate Multiple Myeloma from Waldenström Macroglobulinemia?

Answer 31

Waldenström macroglobulinemia does not have lytic bone lesions, and has proliferation of IgM. Also associated with amyloidosis and hyperviscosity.

Question 32

A solid tumor of plasma cells may occur by 2 ways… name them.

Answer 32

Plasmacytoma

• Solitary plasmacytoma of bone
• Extramedullary plasmacytoma (predilection for the head and neck - nose)

—> do not cause lytic bone lesions

Question 33

Pathology shows:
Monoclonal proliferation of plasma cells
Production of monoclonal immunoglobulins
No symptoms of multiple myeloma and no end organ damage. can progress to multiple myeloma. What is the pathology at hand?

Answer 33

Monoclonal Gammopathy of Undetermined Significance (MGUS)

Question 34

What is the translocation of AML-M3 (Acute Promyelocytic AML)? What is the treatment?

Answer 34

t (15;17)

Tx: All-trans retinoic acid

Question 35

Patient presents with increased WBC count (30-50k) as a result of a stressful event. This can also be a cause of infection, Down Syndrome, Malignancy, Kawasaki Disease, heat stroke, or congenital abnormalities T of Fallot). These cells are predominantly neutrophils and labs show a 5-10% band shift. What is the most likely diagnosis??

Answer 35

Leukemoid Reaction

Question 36

What is the clinical consequence of protein C or protein S deficiency?

Answer 36

Hypercoagulable state