1- Anemia + schistocytes in bl smear + thrombocytopenia points towards MAHA Normal FDPs exclude DIC Fever and CNS symptoms favors the diagnosis of TTP over HUS 2- digital gangrene and DVT occurs due to hypercoaguble state Decrease level of ADAMTS13 which breakdown large multimres of vWF leads to large sticky vWF cause PLTs to clump leading to thrombus formation 3- while bleeding symptoms occur approximately in 20% of cases , in our cases PLTs number not severely reduced so there is no bleeding or petichae 4 - explained above
1 -The diagnosis is most probably (TTP) as there is MAHA and neurological abnormalities Renal impairment 2- gangrene and DVT due to micro thrombosis 3- no bleeding as platelet count not significantly reduced (not below 50k) 4- the pathogenesis of disease is there is a deficiency in ADAM TS13 which is responsible for degradation of VWF polymers into small monomers ready for degradation
Q1/ SLE associated with Anti phospholipid syndrome Q2/ Hypercoagulable state may involve veins and arteries causes DVT and Digital gangrene respectively. Q3/ Because the platelets count is still above 100,000/cmm Q4/ Autoimmune mechanism due to Antiphosphplipid antibodies (lupus anticoagulant, anticardiolipin and anti beta 2 glycoprotein I) والله أعلم
Q1/primary antiphospholipid syndrome Q2/due to multiple thromboembolic evets that result from inactivation of natural anticoagulants as protein C&S and activation of coagulation facrors Q3/ due to prothrombotic effect of antiphospholipid ABs Also presense of FDPs whithin normal range exclude DIC Q4/formation of ABs as lupus anticoagulant and anti cardiolipin againest cell membrane phospholipid forming complexes result in inactivation of natural anticoagulants and activation of coagulation factors
Number 1 : Antiphospholipid antibody syndrome Number 2: its a hypercoagulable state that involve BOTH veins and arteries Number 3 : its a procoagulant state , also the bleeding usually occur when the platelets are with a lower number than 100 K Number 4 : formation of procoagulatory antiphospholipids antibodies which lead to activation of platelets
TTP Anti phospholipid syndrome secondary to SLE Thormboctopenia TTP ( mental confusion . Hemolytic anemia with presence of many fragemented RBCS . Renal impairment . Most probably secondary to SLE . Subcide . DVT . Digital gangrane ( small vessel vasculitis ) secondary to Systemic Lupus
Q1 / Disseminated intravascular coagulation Q2 / because of immobilization because she had stroke before 2 weeks . Q3/ Because the disease in the early stage. Q4 / I don't remember and not sure for these answers , Thank you
1) SLE ( lupus Nephritis and vasculitis) Due to ( sex female , autoimmune hemolytic anemia , hypercoagulable state , mild thrombocytopenia...) 2) Due to thrombosis and vascultits 3) Due to ( hypercoagulability predominance , mild decrease in platelets, normal clotting factor ) 4) Autoimmune mechanism ( genetic predisposition HLA DR2 and 3) + environmental factors Hormonal ( estrogen may have a role ) , autoantibody ( ANA and anti smith)
Question number one answer: Thrombotic thrombocytopenic purpura (TTP) Question number two answer: due to platelet aggregation forming microthrombi that occludes blood vessels and due to hypercoagulability Question number three answer: •TTP primarily affecting platelet dysfunction •Intact coagulation profile Unlike (DIC) PT and PPT are typically normal in (TTP) •Platelet count not significantly low Question number four answer: ADAMTS13 inhibition leading to excessive vWF hence platelet aggregation
1 -Diagnosis is (TTP) based on neurological signs, fever, digital gangrene, DVT, thrombocytopenia, and renal dysfunction. 2-Digital Gangrene & DVT caused by microvascular thrombosis and hypercoagulable state in TTP, Reflects excessive platelet aggregation and microthrombi formation. 3- Because of low platalets are consumed in microthrombi formation rather than causing bleeding and Thrombosis predominates over hemorrhage. 4- Pathogenesis is accumulation of large von Wilebrand factor multimers and excessive platelet aggregation and microthrombi formation which lead to RBC destruction, platelet consumption, and renal dysfunction.
1- Thrombosis + low plateletes + anemia (schistocytes) + high creatinine + CNS affection = Thrombotic thrombocytopenic purpra (TTP) . 2- Digital gangrene and DVT is due to thrmobus formation. 3- There is no bleeding because the plateletes count is above 100,000 (platelets count above 100,000 = asymptomatic thrombocytopenia), "the low plateletes count is due to consumption of platelets in thrombus formation". 4- The pathogenesis of TTP : mutation in ADAMS 13 >> accumulation of large vwf molecules in endothelium >> thrombus formation.
Female with Triad of thrombocytopenia ( decreased platelets) + microangiopathic hemolytic anemia + Acute kidney injury ( high Cr) could be TTP or HUS but presence of (stroke and hallucinations) neurologic symptoms + fever marks the diagnosis of TTP normal PT and PTT >> coagulation pathway is not involved Inhibition of ADAMTS13 level >> uncleaved vWF multimers >> platelet trapping & activation >> microthrombi formation and prothrombotic state. As there is not widespread activation of the clotting factors and FDP is normal unlike DIC >> no consumption of clotting factors >> no bleeding intact platelet function Treatment with plasma exchange + corticosteroids + rituximab + caplasizumab
Most probably diagnosis is Acquired thrombotic thrombocytopenic purpra due to presence of (thrombocytopenia + microangiopathic hemolytic anemia ) , neurological manifestations, fever and kidney affection. It is classic pentad for TTP. Causes of gangrene and DVT Due to formation of microthrombi mainly by platlets Causes of absence of bleeding Due to mild thrombocytopenia Pathogensis May be inherited or acquired but in this case it is acquired Inhibitors of ADAMTS13 enzyme which results in presence of VWF Multimers ( more active ) VWF multimers induce platelet microthrombi formation which cause consumption of platelets ( thrombocytopenia ), fragmented RBCs ( shistocytes ), which are the major criteria for diagnosis Also fever, AKI, and neurological manifestation could be present to microthrombi and ischemia which cause multi organ damage.
TTP Hypercoagulability microthrombi formation Characterized microangiopathic hemolytic anemia and fever and thrombocytopenia >100000 appear in mild renal problems creatinine 1.9 and neurological dysfunction Deficiency of ADAMTS13 Caused by failure to cleave vWF explain the absence of bleeding because Instead, they can cause damage to organs and tissues by blocking blood
Most probably diagnosis is Acquired thrombotic thrombocytopenic purpra due to presence of (thrombocytopenia + microangiopathic hemolytic anemia ) , neurological manifestations, fever and kidney affection. It is classic pentad for TTP. Causes of gangrene and DVT Due to formation of microthrombi mainly by platlets Causes of absence of bleeding Due to mild thrombocytopenia Pathogensis May be inherited or acquired but in this case it is acquired Inhibitors of ADAMTS13 enzyme which results in presence of VWF Multimers ( more active ) VWF multimers induce platelet microthrombi formation which cause consumption of platelets ( thrombocytopenia ), fragmented RBCs ( shistocytes ), which are the major criteria for diagnosis Also fever, AKI, and neurological manifestation could be present to microthrombi and ischemia which cause multi organ damage.
1- Anemia + schistocytes in bl smear + thrombocytopenia points towards MAHA
Normal FDPs exclude DIC
Fever and CNS symptoms favors the diagnosis of TTP over HUS
2- digital gangrene and DVT occurs due to hypercoaguble state
Decrease level of ADAMTS13 which breakdown large multimres of vWF leads to large sticky vWF cause PLTs to clump leading to thrombus formation
3- while bleeding symptoms occur approximately in 20% of cases , in our cases PLTs number not severely reduced so there is no bleeding or petichae
4 - explained above
1 -The diagnosis is most probably (TTP) as there is MAHA and neurological abnormalities Renal impairment
2- gangrene and DVT due to micro thrombosis
3- no bleeding as platelet count not significantly reduced (not below 50k)
4- the pathogenesis of disease is there is a deficiency in ADAM TS13 which is responsible for degradation of VWF polymers into small monomers ready for degradation
1/lupus vasculitis
2/inflammatory mediators , procagulants
3/as autoimmune disese cause micro angiopathic hemolytic anemia (MAHA)
4/lupus vasculitis causes psychogenic manifestions and induce inflammation causes stroke and dvt and autoimmune reaction causes MAHA and affect kidney
Q1/
SLE associated with Anti phospholipid syndrome
Q2/
Hypercoagulable state may involve veins and arteries causes DVT and Digital gangrene respectively.
Q3/
Because the platelets count is still above 100,000/cmm
Q4/
Autoimmune mechanism due to Antiphosphplipid antibodies (lupus anticoagulant, anticardiolipin and anti beta 2 glycoprotein I)
والله أعلم
Q1/primary antiphospholipid syndrome
Q2/due to multiple thromboembolic evets that result from inactivation of natural anticoagulants as protein C&S and activation of coagulation facrors
Q3/ due to prothrombotic effect of antiphospholipid ABs
Also presense of FDPs whithin normal range exclude DIC
Q4/formation of ABs as lupus anticoagulant and anti cardiolipin againest cell membrane phospholipid forming complexes result in inactivation of natural anticoagulants and activation of coagulation factors
Number 1 : Antiphospholipid antibody syndrome
Number 2: its a hypercoagulable state that involve BOTH veins and arteries
Number 3 : its a procoagulant state , also the bleeding usually occur when the platelets are with a lower number than 100 K
Number 4 : formation of procoagulatory antiphospholipids antibodies which lead to activation of platelets
TTP
Anti phospholipid syndrome secondary to SLE
Thormboctopenia
TTP ( mental confusion . Hemolytic anemia with presence of many fragemented RBCS . Renal
impairment . Most probably secondary to SLE . Subcide . DVT . Digital gangrane ( small vessel vasculitis ) secondary to Systemic Lupus
Q1 / Disseminated intravascular coagulation
Q2 / because of immobilization because she had stroke before 2 weeks .
Q3/ Because the disease in the early stage.
Q4 / I don't remember and not sure for these answers , Thank you
1) SLE ( lupus Nephritis and vasculitis)
Due to ( sex female , autoimmune hemolytic anemia , hypercoagulable state , mild thrombocytopenia...)
2) Due to thrombosis and vascultits
3) Due to ( hypercoagulability predominance , mild decrease in platelets, normal clotting factor )
4) Autoimmune mechanism ( genetic predisposition HLA DR2 and 3) + environmental factors
Hormonal ( estrogen may have a role ) , autoantibody ( ANA and anti smith)
Question number one answer:
Thrombotic thrombocytopenic purpura (TTP)
Question number two answer:
due to platelet aggregation forming microthrombi that occludes blood vessels and due to hypercoagulability
Question number three answer:
•TTP primarily affecting platelet dysfunction
•Intact coagulation profile Unlike (DIC) PT and PPT are typically normal in (TTP)
•Platelet count not significantly low
Question number four answer:
ADAMTS13 inhibition leading to excessive vWF hence platelet aggregation
1 -Diagnosis is (TTP) based on neurological signs, fever, digital gangrene, DVT, thrombocytopenia, and renal dysfunction.
2-Digital Gangrene & DVT caused by microvascular thrombosis and hypercoagulable state in TTP,
Reflects excessive platelet aggregation and microthrombi formation.
3- Because of low platalets are consumed in microthrombi formation rather than causing bleeding and Thrombosis predominates over hemorrhage.
4- Pathogenesis is accumulation of large von Wilebrand factor multimers and excessive platelet aggregation and microthrombi formation which lead to
RBC destruction, platelet consumption, and renal dysfunction.
1- Thrombosis + low plateletes + anemia (schistocytes) + high creatinine + CNS affection = Thrombotic thrombocytopenic purpra (TTP) .
2- Digital gangrene and DVT is due to thrmobus formation.
3- There is no bleeding because the plateletes count is above 100,000 (platelets count above 100,000 = asymptomatic thrombocytopenia), "the low plateletes count is due to consumption of platelets in thrombus formation".
4- The pathogenesis of TTP : mutation in ADAMS 13 >> accumulation of large vwf molecules in endothelium >> thrombus formation.
Waiting...
Female with Triad of thrombocytopenia ( decreased platelets) + microangiopathic hemolytic anemia + Acute kidney injury ( high Cr) could be TTP or HUS
but presence of (stroke and hallucinations) neurologic symptoms + fever marks the diagnosis of TTP
normal PT and PTT >> coagulation pathway is not involved
Inhibition of ADAMTS13 level >> uncleaved vWF multimers >> platelet trapping & activation >> microthrombi formation and prothrombotic state.
As there is not widespread activation of the clotting factors and FDP is normal unlike DIC >> no consumption of clotting factors >> no bleeding
intact platelet function
Treatment with plasma exchange + corticosteroids + rituximab + caplasizumab
Most probably diagnosis is Acquired thrombotic thrombocytopenic purpra due to presence of (thrombocytopenia + microangiopathic hemolytic anemia ) , neurological manifestations, fever and kidney affection.
It is classic pentad for TTP.
Causes of gangrene and DVT
Due to formation of microthrombi mainly by platlets
Causes of absence of bleeding
Due to mild thrombocytopenia
Pathogensis
May be inherited or acquired but in this case it is acquired
Inhibitors of ADAMTS13 enzyme which results in presence of VWF Multimers ( more active )
VWF multimers induce platelet microthrombi formation which cause consumption of platelets ( thrombocytopenia ), fragmented RBCs ( shistocytes ), which are the major criteria for diagnosis
Also fever, AKI, and neurological manifestation could be present to microthrombi and ischemia which cause multi organ damage.
TTP
Hypercoagulability Microthrombi formation
Platelets count above 100,000
Inhibition or deficiency of ADAMTS13
No Petechie
anti phospholibid syndrome 2 hypercogulable state with paradoxical plateletes def 3platelt more than 100000 4 form of anti phospholipid anti body
TTP
Hypercoagulability microthrombi formation
Characterized microangiopathic hemolytic anemia and fever
and thrombocytopenia >100000
appear in mild renal problems creatinine 1.9
and neurological dysfunction
Deficiency of ADAMTS13
Caused by failure to cleave vWF
explain the absence of bleeding because Instead, they can cause damage to organs and tissues by blocking blood
Most probably diagnosis is Acquired thrombotic thrombocytopenic purpra due to presence of (thrombocytopenia + microangiopathic hemolytic anemia ) , neurological manifestations, fever and kidney affection.
It is classic pentad for TTP.
Causes of gangrene and DVT
Due to formation of microthrombi mainly by platlets
Causes of absence of bleeding
Due to mild thrombocytopenia
Pathogensis
May be inherited or acquired but in this case it is acquired
Inhibitors of ADAMTS13 enzyme which results in presence of VWF Multimers ( more active )
VWF multimers induce platelet microthrombi formation which cause consumption of platelets ( thrombocytopenia ), fragmented RBCs ( shistocytes ), which are the major criteria for diagnosis
Also fever, AKI, and neurological manifestation could be present to microthrombi and ischemia which cause multi organ damage.