Fatigue - Hubbard Flashcards
what are good questions to ask a possible hematology patient experiencing fatigue?
- fever, night sweats
- bleeding (GI, GU, mucocutaneous)
- LN swelling
- early satiety, weight loss
- prior history of treatment for cancer
- dyspnea
what is hemarthrosis?
- bleeding into joint
- may be from trauma
- common in severe hemophilia
this PE finding suggests either connective tissue disease or hereditary hemorrhagic disease
telangiectasia
what is the most important lab to order for a pt with anemia?
reticulocyte count
what can be detected from a reticulocyte count lab?
- proliferation/stem cell defects
- defects of erythroid progenitor cells
- disturbance of DNA synthesis
- disturbance of hemoglobin synthesis
microcytic, hypochromic anemia (may be normocytic)
- decreased serum iron and ferretin
- increased total iron binding capacity (transferrin less saturated with iron)
iron deficiency anemia
what is the therapy for anemia of chronic disease?
- supportive, if mild
- transfusions may be helpful if anemia is severe
- iron supplementation NOT effective
severe relapsing anemia due to repeated self-induced hemorrhages
- affected pts have underlying psychiatric problems
- tx of underlying dz results in resolution of practice
Lasthenie de Ferjol syndrome
- “facticious anemia”
- iron deficiency anemia
what is the most common disorder caused by a membrane defect leading to increased RBC produciton?
hereditary spherocytosis
what is the most common disorder due to an enzyme deficiency leading to increased RBC production?
G6PD deficiency
what are the most common disorders caused by infections leading to increased RBC production?
malaria, babesia, bartonella
what is the most common disorder caused by infections leading to increased RBC production?
autoimmune hemolysis
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
mechanical/traumatic anemia
- aka march hemoglobinuria, sports anemia, footstrike hemolysis
pts with severe aortic stenosis and pts with prosthetic valves may have ongoing lysis of RBCs
- anemia is usually mild
cardiac anemia
normochromic normocytic anemia
- blood smear shows Heinz bodies (denatured hemoglobin) and bite cells (denatured RBCs with no Hb, removed in the spleen)
G6PD deficiency
recognized as cause of hemolysis for long time
- interferes with cation pump -> shortened RBC survival time
lead toxicity
- lead slows production of RBCs in marrow
MCC hemolytic anemia in the world
- hemolysis can be severe
- urine can contain significant Hb and color can be very dark
malaria
- tx: antimalarial agents and supportive care
MC form of autoimmune hemolytic anemia
- presence of autoantibodies that destroy RBCs at temperatures equal to or greater than normal body temperature
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
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
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!
what is the usual cause of death in treated PCV pts?
progressive marrow fibrosis with pancytopenia
- aka “spent phase” polycythemia
these should be on your DDX for what:
- hemoconcentration
- pulmonary disease (COPD)
- EPO producing tumors
- hemoglobinopathy w/high affinity hemoglobin
PCV
what lung tests do you need to run in pt with PCV?
pulse ox with ABC
- PFT w/ DLCO (CO diffusion)
what enzyme def is seen in over 95% of pts with PCV?
JAK mutation
- with normal serum EPO level, correctly identifies 98% of all PCV pts
the tx for PCV is lowering RBC mass to avoid hyper-viscosity complications
- how is this done?
phlebotomy (250-500cc whole blood ever 1-2 weeks)
- can also be accomplished by hydroxyurea (500-1500mg/d)
