Fatigue - Hubbard

Question 1

what are good questions to ask a possible hematology patient experiencing fatigue?

Answer 1

• fever, night sweats
• bleeding (GI, GU, mucocutaneous)
• LN swelling
• early satiety, weight loss
• prior history of treatment for cancer
• dyspnea

Question 2

what is hemarthrosis?

Answer 2

• bleeding into joint
• may be from trauma
• common in severe hemophilia

Question 3

this PE finding suggests either connective tissue disease or hereditary hemorrhagic disease

Answer 3

telangiectasia

Question 4

what is the most important lab to order for a pt with anemia?

Answer 4

reticulocyte count

Question 5

what can be detected from a reticulocyte count lab?

Answer 5

• proliferation/stem cell defects
• defects of erythroid progenitor cells
• disturbance of DNA synthesis
• disturbance of hemoglobin synthesis

Question 6

microcytic, hypochromic anemia (may be normocytic)

• decreased serum iron and ferretin
• increased total iron binding capacity (transferrin less saturated with iron)

Answer 6

iron deficiency anemia

Question 7

what is the therapy for anemia of chronic disease?

Answer 7

• supportive, if mild
• transfusions may be helpful if anemia is severe
• iron supplementation NOT effective

Question 8

severe relapsing anemia due to repeated self-induced hemorrhages

• affected pts have underlying psychiatric problems
• tx of underlying dz results in resolution of practice

Answer 8

Lasthenie de Ferjol syndrome

• “facticious anemia”
• iron deficiency anemia

Question 9

what is the most common disorder caused by a membrane defect leading to increased RBC produciton?

Answer 9

hereditary spherocytosis

Question 10

what is the most common disorder due to an enzyme deficiency leading to increased RBC production?

Answer 10

G6PD deficiency

Question 11

what are the most common disorders caused by infections leading to increased RBC production?

Answer 11

malaria, babesia, bartonella

Question 12

what is the most common disorder caused by infections leading to increased RBC production?

Answer 12

autoimmune hemolysis

Question 13

this anemia was originally described in soldiers who developed hemoglobinuria after long marches (also common in marathon runners)
- feet striking the ground caused damage/lysis of RBCs in capillaries on plantar surface of feet

Answer 13

mechanical/traumatic anemia

- aka march hemoglobinuria, sports anemia, footstrike hemolysis

Question 14

pts with severe aortic stenosis and pts with prosthetic valves may have ongoing lysis of RBCs
- anemia is usually mild

Answer 14

cardiac anemia

Question 15

normochromic normocytic anemia
- blood smear shows Heinz bodies (denatured hemoglobin) and bite cells (denatured RBCs with no Hb, removed in the spleen)

Answer 15

G6PD deficiency

Question 16

recognized as cause of hemolysis for long time

- interferes with cation pump -> shortened RBC survival time

Answer 16

lead toxicity

- lead slows production of RBCs in marrow

Question 17

MCC hemolytic anemia in the world

• hemolysis can be severe
• urine can contain significant Hb and color can be very dark

Answer 17

malaria

- tx: antimalarial agents and supportive care

Question 18

MC form of autoimmune hemolytic anemia

- presence of autoantibodies that destroy RBCs at temperatures equal to or greater than normal body temperature

Answer 18

warm antibody hemolysis

• steroids are main tx, use in high doses
• transfusion may be helpful (trouble w/crossmatching, watch for signs of hemolysis)
• immunosuppressive may be helpful (cyclophosphamide and azathioprine are most favored)
• splenectomy last resort

Question 19

sx may be insidious and chronic as can PE findings

• facial rubor
• splenomegaly
• HA, dizziness, blurred vision, heaviness in arms/legs
• pruritis after hot shower/bath

Answer 19

PCV

• without tx, 50% mortality at 18 months
• increased risk thrombotic and hemorrhagic complications
• linked to Budd-Chiari syndrome

with tx -> pt can live years/decades!

Question 20

what is the usual cause of death in treated PCV pts?

Answer 20

progressive marrow fibrosis with pancytopenia

- aka “spent phase” polycythemia

Question 21

these should be on your DDX for what:

• hemoconcentration
• pulmonary disease (COPD)
• EPO producing tumors
• hemoglobinopathy w/high affinity hemoglobin

Answer 21

PCV

Question 22

what lung tests do you need to run in pt with PCV?

Answer 22

pulse ox with ABC

- PFT w/ DLCO (CO diffusion)

Question 23

what enzyme def is seen in over 95% of pts with PCV?

Answer 23

JAK mutation

- with normal serum EPO level, correctly identifies 98% of all PCV pts

Question 24

the tx for PCV is lowering RBC mass to avoid hyper-viscosity complications
- how is this done?

Answer 24

phlebotomy (250-500cc whole blood ever 1-2 weeks)

- can also be accomplished by hydroxyurea (500-1500mg/d)