349) A 46 year old man presents to your office with generalized itching. The itching is severe during shower and for past few weeks, it has become intolerable. He denies any headache, chest pain or shortness of breath. He does not smoke and consumes alcohol only occasionally. He works as a public health officer in New York City. He reports no sleep related problems, denies snoring at night or day-time drowsiness. He denies any past medical problems . On examination, he is overweight, afebrile, Blood pressure 110/60 mm Hg, Heart Rate 88/min, Respiratory rate is 16/minute. Physical examination is benign. There is no splenomegaly. A CT Scan of the chest, abdomen and pelvis does not reveal any neoplasm. Laboratory investigations reveal :
WBC : 9.0k/µl
Hemoglobin 20gm% ( N = 13.5 to 16.5gm%
Hematocrit 62%
Mean Corpuscular Volume ( MCV) 65µl
Platelet count 280k/µl
Serum Creatinine 0.8mg%
Serum Calcium 9.8mg%
Erythropoetin Level 3U/L ( Normal 5 to 25U/L)
Which of the following is features can be seen with this condition?
A) Low serum uric acid
B) Iron deficiency
C) Elevated HGBA2 fraction on HGB electrophoresis
D) Reduced Leucocyte Alkaline Phosphatase
E) Elevated Reticulocyte Count
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It is “C”
C) Elevated HGBA2 fraction on HGB electrophoresis
B) Iron deficiency
Microcytosis – either thalassemia or iron deficiency
But this picture looks like polycythemia
Does polycythemia cause iron deficiency?
Its polycythemia vera and ans is increase uric acid level
I will go with “E”.
Polycythemia Vera
Hg >18 g/dl or Hematocrit >52% in white men
Hg >16 g/dl or Hematocrit >47% in African American and women
Splenomegaly with or without thrombocytosis and leukocytosis
Portal vein thrombosis
Diagnosis depend on the criteria:
3 Major criteria or 2 First Major criteria and 2 Minor criteria out of 4 minor criteria is diagnosis of Primary Polycythemia vera.
Major criteria are:
1) increase red cells mass
2) oxygen sat > 92 %
3) splenomegaly
Minor criteria are:
1) Leukocyte alkaline phosphatase >100 U/L
2) Platelet count > 400,000 per mm3
3) White blood cells > 12,000 per mm3
4) Serum B12 level > 900 pg per ml or serum unbound B12 binding capacity > 2,200 pg per ml
Yes. This is Polycythemia. High Hb, no features of hypoxia, low mcv. (Thalassemia minor can present with low mcv and high rbc mass but Hb level will not be high, because it has problem in producing hemoglobin, so exclude the Thalassemia here. Iron Deficiency is also ruled out because it does not have anemia. Polycythemia can present with iron deficieny when it was treated with repeated phlebotomy). Polycythemia doesnt need to increase all cell lines.
Polycythemia can present with pruritus after warm bathe and bone marrow is producing rbc and retic count is moderately high in Polycythemia. That’s what I got.
Answer is E
E
Thalassemia + multiple blood transfusions recently
surely its C- pic is showing polycythemia & its not primary as only Hb is raised.multiple transfusion increased Hb & thus supress erthropoitin which is low in this case.
as the eythropoetin level is supressed hence the haematocrit is raised due to a secondary cause .it is not polycythemia vera as it tends to increase all the cell lines.but as it is microcytic it shld be iron deficiency or thalassemia.hence it is thalassemia.
~E
pt has classic sign of polycythemia vera, post-shower itching.
dr red pls give the answer
i go wth pcv
answer is b/e
since low mcv answer is iron deficiecy
Reticulocytes appear slightly bluer than other red cells when looked at with the normal Romanowsky stain. Reticulocytes are also relatively large, a characteristic that is described by the MCV (mean corpuscular volume)