Pathoma: Hemostasis and Related Disorders Test with Solutions

Pathoma: Hemostasis and Related Disorders Test with
Solutions

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