Thrombotic microangiopathy: Difference between revisions

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Syndrome of microangiopathic hemolytic anemia, thrombocytopenia, and microvascular thrombosis leading to organ dysfunction

Disease	Clinical Clues
Primary
Thrombotic thrombocytopenia purpura	PT/INR and PTT are normal
Hemolytic-uremic syndrome	PT/INR and PTT are normal, severe AKI
Atypical hemolytic-uremic syndrome	PT/INR and PTT are normal, severe AKI
HELLP syndrome	pregnancy
Secondary
Autoimmune disease, including systemic lupus erythematosus or antiphospholid antibody syndrome
Drug-induced thrombotic microangiopathy	including quinine, ticlopidine, and chemotherapy (mitomycin, gemcitabine, cyclosporine, and tacrolimus, though it may also be caused by the underlying condition
Sepsis
Secondary hemolytic-uremic syndrome	most commonly from Streptococcus pneumoniae or influenza
DIC	PT/INR and PTT are prolonged
Malignancy
HIV

Investigations

Confirm presence of TMA: CBC, reticulocyte count, LDH, peripheral blood film, and haptoglobin
Assess for end-organ damage: electrolytes, troponin, lactate, liver enzymes, bilirubin, urinalysis, urea, creatinine, lipase
Assess for TTP: plasma ADAMTS-13 activity ± inhibitor
Assess for infectious causes: stool for culture (for STEC), stool for Shiga toxin (PCR or ELISA, as available), and-LPS antibodies, chest x-ray, blood cultures ± urine ± CSF cultures, multiplex NP swab, HIV serology, hepatitis B and C serology
Assess for other coexisting diseases:
- Lipase, complement C3/C4, ANA, anti-dsDNA, anti-centromere, anti-Scl-70, calcium, INR/PTT, fibrinogen, FDP, D-dimer, lupus anticoagulant, anti-cardiolipin, β2 glycoprotein, DAT/Coombs test
- ANCA, anti-GBM, beta-hCG
Assess for atypical HUS: tests of complement activation (CFB/Ba/Bb, C5b-9 level, CH50),, anti-CFH antibodies, MCP surface expression, screening for mutations in complement pathway genes

@@ Line 1: / Line 1: @@
 *Syndrome of [[microangiopathic hemolytic anemia]], [[thrombocytopenia]], and microvascular thrombosis leading to organ dysfunction
+{| class="wikitable"
-*Primary
+!Disease
-**[[Thrombotic thrombocytopenia purpura]]
+!Clinical Clues
-**[[Hemolytic-uremic syndrome]]
+|-
-**[[Atypical hemolytic-uremic syndrome]]
+! colspan="2" |Primary
-**[[HELLP syndrome]]
+|-
-*Associated with coexisting condition
+|[[Thrombotic thrombocytopenia purpura]]
-**Autoimmune disease, including [[systemic lupus erythematosus]] or [[antiphospholid antibody syndrome]]
+|PT/INR and PTT are normal
-**[[Drug-induced thrombotic microangiopathy]], including [[quinine]], [[ticlopidine]], and [[chemotherapy]] ([[mitomycin]], [[gemcitabine]], [[cyclosporine]], and [[tacrolimus]], though it may also be caused by the underlying condition)
+|-
-**[[Sepsis]] and [[secondary hemolytic-uremic syndrome]] (usually from [[Streptococcus pneumoniae]] or [[influenza]])
+|[[Hemolytic-uremic syndrome]]
-**[[DIC]]
+|PT/INR and PTT are normal, severe AKI
-**[[Malignancy]]
+|-
-**[[HIV]]
+|[[Atypical hemolytic-uremic syndrome]]
+|PT/INR and PTT are normal, severe AKI
+|-
+|[[HELLP syndrome]]
+|pregnancy
+|-
+! colspan="2" |Secondary
+|-
+|Autoimmune disease, including [[systemic lupus erythematosus]] or [[antiphospholid antibody syndrome]]
+|
+|-
+|[[Drug-induced thrombotic microangiopathy]]
+|including [[quinine]], [[ticlopidine]], and [[chemotherapy]] ([[mitomycin]], [[gemcitabine]], [[cyclosporine]], and [[tacrolimus]], though it may also be caused by the underlying condition
+|-
+|[[Sepsis]]
+|
+|-
+|[[Secondary hemolytic-uremic syndrome]]
+|most commonly from [[Streptococcus pneumoniae]] or [[influenza]]
+|-
+|[[DIC]]
+|PT/INR and PTT are prolonged
+|-
+|[[Malignancy]]
+|
+|-
+|[[HIV]]
+|
+|}
+== Investigations ==
+* Confirm presence of TMA: CBC, reticulocyte count, LDH, peripheral blood film, and haptoglobin
+* Assess for end-organ damage: electrolytes, troponin, lactate, liver enzymes, bilirubin, urinalysis, urea, creatinine, lipase
+* Assess for TTP: plasma ADAMTS-13 activity ± inhibitor
+* Assess for infectious causes: stool for culture (for STEC), stool for Shiga toxin (PCR or ELISA, as available), and-LPS antibodies, chest x-ray, blood cultures ± urine ± CSF cultures, multiplex NP swab, HIV serology, hepatitis B and C serology
+* Assess for other coexisting diseases:
+** Lipase, complement C3/C4, ANA, anti-dsDNA, anti-centromere, anti-Scl-70, calcium, INR/PTT, fibrinogen, FDP, D-dimer, lupus anticoagulant, anti-cardiolipin, β2 glycoprotein, DAT/Coombs test
+** ANCA, anti-GBM, beta-hCG
+* Assess for atypical HUS: tests of complement activation (CFB/Ba/Bb, C5b-9 level, CH50),, anti-CFH antibodies, MCP surface expression, screening for mutations in complement pathway genes
+== Further Reading ==
+* Making the Correct Diagnosis in Thrombotic Microangiopathy: A Narrative Review. ''Can J Kidney Health Dis''. 2021;8. doi: [https://doi.org/10.1177/205435812110087 10.1177/205435812110087]
 [[Category:Hematology]]