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Pathoma: Hemostasis and Related Disorders Test with Solutions
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Pathoma: Hemostasis and Related Disorders Test withSolutions
idiopathic thrombocytopenic purpura: IgG Correct Ans-they are produced by plasma cells
in the spleen
then, the Ab bound platelets are alter consumed by the macrophages in the spleen
idiopathic thrombocytopenic purpura: forms Correct Ans-acute
- think children
chronic
- think adults, think ppl with AI disease
idiopathic thrombocytopenic purpura: acute form Correct Ans-children
1. thrombocytopenia weeks after infection or immunization
2. self0limiting d/o within weeks of pt
Tx: support, esp. with really bad thrombocytopenia
idiopathic thrombocytopenic purpura: chronic form Correct Ans-1. usually F of child
bearing age
2. may be primary or secondary
, Pathoma: Hemostasis and Related Disorders Test with
Solutions
idiopathic thrombocytopenic purpura: pregnant women Correct Ans-may cause a short
lived thrombocytopenia in the offspring
0 this is due to the fact that the anti platelet IgG can cross the placenta; this is something to
look out for with women with lupus
idiopathic thrombocytopenic purpura: labs Correct Ans-1. decreased platelets
2. PT/PTT norma; this is bc the coagulation cascade is not affected
3. increase int he megakaryocytes in the bone marrow;
- body senses low platlets
- attempt at compensation
idiopathic thrombocytopenic purpura: Tx Correct Ans-1. corticosteroids
- works hella good in acute
- relapse common in the chrnoic form
2. IVIG:
- short lived tx for serious bleeding concern
- decoy for the splenic macrophages such that they are overwhelmed consuming the IVIG and
are thus less likely to be chomping on an IgG-bound platelet
3. for refractory cases: splenectomy
, Pathoma: Hemostasis and Related Disorders Test with
Solutions
- eliminates both the source of the Ab and the source of the site of destruction
Microangiopathic hemolytic anemia: what it do Correct Ans-formation of small thrombi
that cause stress on RBCs, shearing them and causing the formation of both schistocytes and
anemia
Microangiopathic hemolytic anemia: why talk about it here? Correct Ans-due to the
microthrombi that form (made only of platelets), there is consumption of a lot of platelets
throughout the tissues.
this leads to a thrombocytopenia
schistocyte Correct Ans-helmet cells
Microangiopathic hemolytic anemia: two diseases Correct Ans-1. TTP- thrombotic
thrombocytopenic purpura
2. HUS - hemolytic uremic syndrome
TTP: what it do? Correct Ans-platelet microthrombi form due to a deficiency or decrease in
the amount of Adams Ts protein
TTP: adams ts 13 Correct Ans-protein that is needed to cleave multimers of vWF that are
produced in order to get the active form of this
, Pathoma: Hemostasis and Related Disorders Test with
Solutions
the multimeric form then piles up and leads to abnormal adhesion (Gp1B to vWF) leading to
the microthrombi
TTP: what causes the adams ts 13 decrease Correct Ans-1. can be a genetic defect but
2. autoantibody production against the protein
TTP: who Correct Ans-adult females with an AI process such as lupus
HUS: what is it Correct Ans-endothelial damage to the blood vessels classically by drugs or
infections leads to microthrombi formation
- this leads to consumption of platelets and thrombocytopenia
- this also causes schistocytes to develop
anemia + decrease in the renal blood flow --> risk of renal damage and acute renal failure
HUS: how to get it Correct Ans-children who are exposed to the undercooked beef
- E coli 015787
-- verotoxin leads to endothelial damage resulting in
-- dysentery
-- renal issues primarily
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