Thrombotic Thrombocytopenic Purpura (TTP)MANAGEMENT

HEMATOLOGIC AND ONCOLOGIC PRESENTATIONS · 12 SECTIONS

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720 items

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Cardiovascular Presentations71▾

Respiratory Presentations52▾

Neurological Presentations83▾

Gastrointestinal Presentations74▾

Genitourinary and Reproductive Presentations48▾

Infectious Disease Presentations148▾

Trauma Presentations89▾

Toxicologic Presentations80▾

Psychiatric Presentations26▾

Musculoskeletal and Rheumatologic Presentations96▾

Dermatology Presentations23▾

Environmental and Exposure Presentations27▾

Hematologic and Oncologic Presentations36▾

Acute chest syndrome Acute Hemolytic Transfusion Reaction Anticoagulant reversal (warfarin, DOAC, heparin)Aplastic Anemia (Crisis)Autoimmune Hemolytic Anemia CAR-T and cytokine release syndrome Coagulopathy Delayed Hemolytic Transfusion Reaction Disseminated Intravascular Coagulation (DIC)Essential Thrombocythemia Febrile Non-hemolytic Transfusion Reaction Graft Versus Host Disease (Acute)Hemolytic Uremic Syndrome (HUS)Hemophilia A (Bleeding Crisis)Heparin-Induced Thrombocytopenia (HIT)Hyperviscosity syndrome Immune checkpoint inhibitor toxicity Immune Thrombocytopenic Purpura (ITP)Leukemoid Reaction Leukostasis Massive transfusion protocol Myelofibrosis Neutropenic enterocolitis (typhlitis)Paroxysmal Nocturnal Hemoglobinuria Polycythemia Vera (Crisis)Pure Red Cell Aplasia Sickle Cell (Acute Chest Syndrome)Sickle Cell (Splenic Sequestration)Sickle cell crisis (vaso-occlusive)Symptomatic anemia Thrombocytopenia (severe)Thrombotic Thrombocytopenic Purpura (TTP)Transfusion-Associated Circulatory Overload (TACO)Transfusion-Related Acute Lung Injury (TRALI)Tumor lysis syndrome Von Willebrand Disease (Bleeding)

Pediatric-Specific Presentations19▾

Endocrine and Metabolic Presentations45▾

ENT and Maxillofacial Presentations27▾

Ophthalmologic Presentations19▾

Obstetric Presentations23▾

Systemic and Miscellaneous Presentations17▾

01Approach to the Critical Patient/attcp44

Immediate priorities

›Recognition and resuscitation triggers

›TTP pentad: MAHA + thrombocytopenia + neurologic dysfunction + renal dysfunction + fever

›Fewer than 10% present with all five features; treat empirically when MAHA + thrombocytopenia present

›Untreated mortality approaches 90%

›Emergent hematology consultation for all suspected TTP

›Do not await ADAMTS13 result before initiating treatment

›Class I recommendation, expert consensus

›Transfer immediately to center with therapeutic plasma exchange (TPE) capability if not available

›TPE is life-saving and should not be delayed

›Fresh frozen plasma (FFP) infusion as bridge if TPE unavailable

Monitoring and targets

›Hemodynamic and neurologic monitoring

›Continuous cardiac monitoring

›Arrhythmia risk from cardiac microthrombi

›QT prolongation screen before drugs that prolong QT

›Neurologic checks every 2 to 4 hours

›GCS scoring: GCS <= 14 associated with 9-fold increase in mortality

›Seizure precautions for altered consciousness

›Daily CBC and LDH until platelet count stabilizing

›Platelet count doubling after first TPE is favorable response marker

›LDH trend reflects microthrombus burden

›Troponin monitoring

›Elevated in 54 to 68% of patients

›Troponin above ULN associated with 6-fold increase in mortality

Escalation triggers

›Critical escalation criteria

›Seizures or declining GCS

02History/hx77

Presenting symptoms

›Neurologic symptoms (most common: 39 to 80% of cases)

›Headache

›Often severe and diffuse

›Early and common presenting feature

›Confusion or altered mental status

›May fluctuate

›Delirium pattern common

›Visual changes

›Blurring, diplopia, visual field deficits

›Cortical infarction mechanism

›Seizures

›Focal or generalized

›Elevated mortality risk marker

›Focal weakness or speech difficulty

›Stroke-like presentation possible

›Microthrombus-mediated ischemia

›Hematologic and constitutional symptoms

›Petechiae, purpura, easy bruising

›Thrombocytopenia-related

›Mucosal bleeding: gingival, epistaxis

›Fatigue and pallor

›Microangiopathic hemolytic anemia

›Hemoglobin typically 60 to 100 g/L

›Dark urine

›Hemoglobinuria from hemolysis

›Distinguish from hematuria

›Fever (10 to 35% of cases)

03Physical Exam/exam72

Vitals and general appearance

›Vital sign patterns

›Fever (10 to 35%)

›Low-grade most common

›High fever raises infection concern

›Tachycardia

›Anemia-driven compensatory response

›Cardiac microthrombus-mediated arrhythmia

›Blood pressure

›Hypertension from renal microvascular injury

›Hypotension if cardiogenic shock from troponin-positive disease

›Respiratory rate

›Elevated if severe anemia or heart failure

›Oxygen saturation monitoring indicated

Skin and mucosal findings

›Bleeding manifestations

›Petechiae

›Dependent distribution

›Lower extremity, buccal mucosa

›Purpura and ecchymoses

›Non-palpable, flat lesions

›Distinguish from vasculitis (palpable purpura)

›Mucosal bleeding

›Gingival bleeding

›Epistaxis

›Hemolysis signs

›Jaundice or scleral icterus

›Indirect hyperbilirubinemia from hemolysis

04Differential Diagnosis/ddx49

Thrombotic microangiopathy spectrum

›Primary TMA diagnoses

›Immune TTP (iTTP): ICD-10 M31.1

›ADAMTS13 activity < 10% confirms diagnosis

›Autoimmune etiology; anti-ADAMTS13 IgG antibodies

›Congenital TTP (Upshaw-Schulman syndrome): ICD-10 M31.1

›Biallelic ADAMTS13 mutations

›ADAMTS13 activity near zero, no inhibitor detected

›Complement-mediated HUS (aHUS)

›Platelets often > 30 x 10^9/L

›Creatinine typically > 177 mmol/l

›ADAMTS13 activity normal or mildly reduced

›Shiga toxin-associated HUS (STEC-HUS)

›Bloody diarrhea prodrome

›E. coli O157:H7 or other Shiga toxin producers

›More common in children

Mimics with overlapping hematologic features

›Hematologic mimics

›Disseminated intravascular coagulation (DIC): ICD-10 D65

›Prolonged PT/aPTT and low fibrinogen

›Elevated D-dimer

›Underlying sepsis, malignancy, or obstetric emergency

›Evans syndrome

›Positive direct antiglobulin test (DAT)

›Autoimmune hemolytic anemia plus immune thrombocytopenia

›ADAMTS13 activity normal

›Severe vitamin B12 deficiency

›MCV elevated (mean 109 fL)

05Laboratory Tests/lt83

Essential diagnostic labs

›Peripheral blood smear

›Schistocytes (fragmented red cells)

›Threshold > 1% for significant MAHA

›May be absent on first smear; repeat daily

›Thrombocytopenia

›Typically < 30 x 10^9/L in iTTP

›Low platelet count from consumption in microthrombi

›Polychromasia and nucleated red cells

›Reflect hemolytic stress

›Elevated reticulocyte count expected

›Hemolysis panel

›LDH (lactate dehydrogenase)

›Markedly elevated reflecting RBC destruction and tissue ischemia

›LDH > 10x ULN is independent predictor of 30-day mortality

›Serial trending guides response to TPE

›Indirect bilirubin

›Elevated from hemoglobin catabolism

›Direct bilirubin normal (hepatic function preserved)

›Haptoglobin

›Undetectable in active hemolysis

›Very sensitive marker for intravascular hemolysis

›Reticulocyte count

›Elevated in compensated hemolysis

›Low in early or marrow-suppressed disease

Confirmatory and ruling-out labs

›ADAMTS13 activity assay

›Draw before initiating TPE if possible

›TPE replaces ADAMTS13 and confounds assay

›Rapid assay available at some centers (turnaround 2 to 4 hours)

›Activity < 10% confirms iTTP

›Sensitivity 89 to 96% for diagnosing iTTP

›Specificity approaches 95% at < 10% threshold

›Activity 10 to 20% borderline; clinical correlation required

›May still represent iTTP or congenital TTP

›Anti-ADAMTS13 antibody testing concurrent

›Anti-ADAMTS13 antibody testing

›IgG ELISA and functional inhibitor (Bethesda) assay

›Confirms immune etiology when positive

›Negative in congenital TTP and drug-induced TMA

›Guides duration of rituximab therapy

›Antibody titer correlates with relapse risk

›Monitoring every 3 to 6 months in remission

›Direct antiglobulin test (DAT)

›Negative in TTP (distinguishes from autoimmune hemolytic anemia)

›Positive DAT requires Evans syndrome consideration

›TTP and Evans can rarely coexist

Organ injury and baseline labs

›Cardiac biomarkers

›Troponin (I or T)

›Elevated in 54 to 68% of patients

›cTnI > 0.25 microg/L associated with 3-fold increase in death or refractoriness

›Troponin elevation from cardiac microthrombus-mediated ischemia

›NT-proBNP if heart failure features

›Assess for volume overload from TPE (FFP administration)

›Cardiac dysfunction prognostic marker

›Renal function

›Creatinine and eGFR

›Creatinine typically < 177 mmol/l in iTTP (distinguishes from aHUS)

›Severe renal failure (< 5% of iTTP) suggests alternative TMA

›Urinalysis

›Hemoglobinuria (dark urine without red cells on microscopy)

›Protein and red cell casts if renal parenchyma involved

›Coagulation studies

›PT/INR and aPTT typically normal or mildly prolonged

›Normal coagulation distinguishes from DIC

›Fibrinogen normal or elevated

›D-dimer

›Mildly elevated in TTP; markedly elevated favors DIC

›Useful differentiating test

›Infection and immune workup

›HIV testing (all patients)

›HIV-associated TTP has distinct management implications

›Required before rituximab

›Hepatitis B surface antigen and core antibody

›Required before rituximab to prevent reactivation

›Antiviral prophylaxis if HBsAg positive

›ANA, antiphospholipid antibodies

›Systemic autoimmune disease evaluation

›Guides long-term immunosuppression decisions

›Pregnancy test (all women of childbearing age)

›Pregnancy-associated TTP distinct management

›Affects drug safety profile

›Blood cultures if febrile

›Exclude bacterial sepsis

›Sepsis and TTP can coexist

06Diagnostic Tests/dt87

Scoring Systems

›PLASMIC score

›Seven-variable validated prediction tool for ADAMTS13 < 10%

›Platelet count < 30 x 10^9/L: 1 point

›Combined hemolysis variable (reticulocyte > 2.5%, haptoglobin undetectable, or indirect bilirubin > 34 mmol/l): 1 point

›No active cancer in past year: 1 point

›No solid organ or stem cell transplant: 1 point

›MCV < 90 fL: 1 point

›INR < 1.5: 1 point

›Creatinine < 177 mmol/l: 1 point

›Score interpretation

›Score 0 to 4 (low risk): ADAMTS13 < 10% in 0% of patients

›Score 5 (intermediate risk): ADAMTS13 < 10% in 5 to 24% of patients

›Score 6 to 7 (high risk): ADAMTS13 < 10% in 72 to 82% of patients

›Diagnostic performance: AUROC 0.96 in meta-analysis (Paydary et al., Transfusion 2020)

›Score >= 5 warrants empiric TPE initiation before ADAMTS13 result

›Class I expert consensus recommendation

›French Thrombotic Microangiopathies score (FTM)

›Alternative scoring tool used in European centers

›Incorporates similar clinical and laboratory variables

›Less validated than PLASMIC in external cohorts

›PLASMIC preferred in North American practice

MRI

›Brain MRI indications

›Neurologic symptoms with normal or equivocal CT head

›More sensitive than CT for cortical and subcortical ischemia

›Diffusion-weighted imaging detects acute ischemic foci

›Multiple punctate infarcts in different vascular territories

›Microthrombus-mediated embolic pattern

›Differentiates from large vessel stroke

›Posterior reversible encephalopathy syndrome (PRES) pattern

›White matter changes on FLAIR

›Associated with hypertension and TMA

›MRI brain findings in TTP

›Small cortical and subcortical infarcts

›Bilateral, multifocal distribution

›Evolve over hours to days

›FLAIR hyperintensities

›Restricted diffusion on DWI

›May normalize with recovery

›MRI contraindications

›Hemodynamically unstable patient

›Non-MRI-compatible devices or implants

›CT head as first-line for rapid exclusion of hemorrhage

›CT head without contrast

›Indications

›Focal neurologic deficits or new seizures

›GCS < 14 or declining mental status

›Exclude intracranial hemorrhage before anticoagulation decisions

›Expected findings

›Often normal in early TTP (ischemia not yet visible)

›Hemorrhagic transformation of ischemic areas possible

›CT head limitations

›Low sensitivity for acute ischemia within 6 hours

›MRI preferred for parenchymal detail if stable

›CT abdomen-pelvis

›Indications

›Severe abdominal pain with peritoneal signs

›Exclude bowel ischemia if mesenteric microthrombi suspected

›Alternative TMA diagnosis exclusion (malignancy, pancreatitis)

›Contrast considerations

›Renal function assessment required

›Low-dose CT without contrast preferred if creatinine elevated

Ultrasound

›Echocardiography (cardiac ultrasound)

›Indications

›Troponin elevation in any TTP patient

›Clinical heart failure or cardiogenic shock

›Dysrhythmia or new ECG changes

›Expected findings

›Regional wall motion abnormalities from microthrombi

›Reduced ejection fraction in severe cardiac involvement

›Pericardial effusion (rare but described)

›Prognostic value

›Reduced EF correlates with mortality risk

›Serial echo guides fluid management during TPE

›Abdominal ultrasound

›Splenomegaly assessment

›Uncommon in acute TTP

›Prior splenectomy status affects immunologic response

›Hepatic assessment if liver disease suspected

›Exclude chronic liver disease causing coagulopathy

›Portal hypertension screen

›Point-of-care ultrasound (POCUS)

›Volume status during TPE

›IVC collapsibility to guide FFP infusion rate

›Pulmonary B-lines for pulmonary edema from volume

›Pleural effusion screen

›Transudative effusions from cardiac dysfunction

›Exudative effusions raise infection concern

07Disposition/dispo34

Admission and level of care

›All suspected TTP requires hospital admission

›No safe outpatient management with active MAHA plus thrombocytopenia

›ICU or high-acuity monitoring unit with apheresis capability

›ISTH 2025 guideline Class I recommendation

›Transfer to TPE-capable center if not locally available

›Bridge with FFP infusion (25 to 30 mL/kg) during transfer

›Coordinate emergently; delays increase mortality

›ICU admission criteria

›Neurologic deterioration

›GCS < 14 or declining GCS

›Active seizures

›Cardiac involvement

›Troponin elevation with hemodynamic instability

›Heart failure or arrhythmia

›Hemodynamic instability

›SBP < 90 mmHg

›Shock requiring vasopressors

Discharge criteria

›Criteria for hospital discharge

›Platelet count sustained >= 150 x 10^9/L for 2 or more consecutive days

›Declining LDH trend toward normal

›Resolution of active hemolysis (rising haptoglobin, normalizing bilirubin)

›No new neurologic symptoms for 48 to 72 hours

›Stable or improving mental status

›No recurrence of seizures off prophylactic treatment

›TPE safely discontinued

›Hematology clearance required

08Treatment/t83

Emergency initiation of TPE

›Therapeutic plasma exchange (TPE)

›Start within 4 to 8 hours of diagnosis in high-suspicion cases

›Do not await ADAMTS13 result if PLASMIC score >= 5

›Class I, ISTH 2025 guideline

›Volume and schedule

›1 to 1.5 plasma volumes per session daily

›Continue daily until platelet count >= 150 x 10^9/L for 2 consecutive days

›Replacement fluid

›Fresh frozen plasma (FFP) preferred replacement

›FFP supplies functional ADAMTS13 and removes inhibitors

›Refractory or relapsing disease escalation

›Escalate to twice-daily TPE after 5 sessions without response

›Add rituximab and caplacizumab if not already initiated

Corticosteroids

›Corticosteroid therapy

›Standard dosing: prednisone 1 mg/kg/day orally

›Initiate with first TPE session

›Continue until platelet count normalizes

›High-dose pulse for severe or refractory disease

›Methylprednisolone 1000 mg IV daily for 3 days

›Indicated for severe neurologic involvement or cardiac dysfunction

›JAMA 2025 evidence level: expert consensus recommendation

›Taper guidance

›Gradual taper over 4 to 6 weeks after remission

›Faster taper if rituximab co-administered

Rituximab

›Rituximab (anti-CD20 monoclonal antibody)

›Dosing regimen

›375 mg/m^2 IV weekly for 4 doses

›Alternative: 1000 mg IV twice (days 1 and 15) in some protocols

›Timing of initiation

›Can be given from day 1 in severe or high-risk cases

›If normal response to TPE: initiate within first 2 weeks

›Pre-rituximab requirements

›HIV testing before administration

›Hepatitis B screening (HBsAg, anti-HBc); antiviral prophylaxis if positive

›Avoid live vaccines for 12 months post-treatment

›Benefit

›Reduces relapse rate; first-line adjunct with TPE

›Reduces 30-day mortality when added to TPE plus steroids

›Preemptive rituximab for ADAMTS13 relapse: OR 0.09 for clinical relapse

Caplacizumab

›Caplacizumab (anti-vWF Nanobody)

›Mechanism

›Blocks interaction of ultra-large vWF with platelet GPIb-alpha

›Reduces platelet-rich microthrombus formation

›Dosing (FDA-approved, 2019)

›11 mg IV bolus before first TPE session

›Then 11 mg subcutaneously once daily for duration of TPE

›Continue 11 mg SC daily for at least 30 days after last TPE session

›Clinical benefit (HERCULES trial)

›Faster time to platelet normalization

›Reduced refractoriness and TTP-related death vs. placebo

›Higher rate of TTP exacerbation after discontinuation if ADAMTS13 not recovered

›Monitoring

›ADAMTS13 activity before stopping; if still < 10%, extend duration

›Monitor for bleeding: mucosal, minor; serious bleeding uncommon

Contraindicated and avoided treatments

›Platelet transfusion

›Generally avoided in TTP

›Theoretical risk of fueling microvascular platelet thrombi

›Observational data suggest increased thrombotic events

›Exception: life-threatening hemorrhage (intracranial, gastrointestinal)

›Transfuse only when bleeding risk exceeds thrombosis risk

›Hematology guidance required

›Drug avoidance

›Re-exposure to suspected causative drug absolutely contraindicated

›Quinine: even trace amounts in beverages

›Document drug-TMA in allergy system

›Avoid antiplatelet agents acutely unless specific cardiac indication

›Aspirin and clopidogrel increase bleeding risk

›Anticoagulation not routinely indicated

Refractory TTP management

›Salvage therapies

›Cyclosporine

›2 to 3 mg/kg/day PO in divided doses

›Target trough level 100 to 150 ng/mL

›Renal function monitoring required

›Bortezomib (proteasome inhibitor)

›1.3 mg/m^2 IV or SC on days 1, 4, 8, 11

›Targets plasma cells producing anti-ADAMTS13 antibodies

›Peripheral neuropathy monitoring

›Daratumumab (anti-CD38)

›16 mg/kg IV weekly for refractory iTTP

›Emerging evidence; case series and small trials

›Splenectomy

›Considered for chronic relapsing or immunosuppression-refractory iTTP

›Removes primary site of antibody production and RBC destruction

09Special Populations/sp53

Pregnancy

›Pregnancy-associated TTP

›Epidemiology and timing

›7% of iTTP cases; incidence 1 per 25,000 pregnancies

›Second and third trimester predominance; postpartum cases also occur

›ADAMTS13 deficiency persists through pregnancy but crisis may be triggered

›Distinguishing from HELLP syndrome

›HELLP: hypertension, liver enzyme elevation, resolves with delivery

›TTP: ADAMTS13 < 10%, neurologic predominance, does not resolve with delivery

›Overlap possible; empiric TPE when uncertain in severe MAHA

›Treatment modifications

›TPE is safe in pregnancy; first-line treatment

›Corticosteroids (methylprednisolone or prednisone): generally safe

›Rituximab: avoid in first and second trimester (Category C/teratogenic risk)

›Caplacizumab: limited pregnancy safety data; individualized risk-benefit

›Fetal monitoring

›Continuous electronic fetal monitoring during TPE sessions

›Obstetrics consultation for delivery planning

›Delivery does NOT treat iTTP (contrast with HELLP)

Geriatric

›Older adult considerations

›Atypical presentations

›Baseline cognitive impairment confounds neurologic assessment

›Fatigue and functional decline as primary symptoms

›Lower threshold for TTP workup in unexplained anemia plus thrombocytopenia

›Treatment tolerability

›TPE well-tolerated but higher risk of line complications

›Citrate toxicity (hypocalcemia) more symptomatic; calcium supplementation during TPE

10Background/b72

Epidemiology

›Incidence and demographics

›Annual incidence: 2 to 6 cases per million population

›Higher in females: incidence rate ratio 3.19

›Higher in Black individuals: incidence rate ratio 7.09

›Peak incidence in adults aged 30 to 50 years

›Mortality without treatment

›Untreated mortality approaches 90%

›With triple therapy (TPE + steroids + rituximab), 30-day survival > 93%

›Relapse epidemiology

›16% clinical relapse rate at 5 years

›30 to 40% combined clinical or ADAMTS13 relapse at 5 years

›Preemptive rituximab reduces relapse to near zero in high-risk patients

›Associated conditions distribution

›Idiopathic (no identifiable trigger): largest subgroup

›Autoimmune mechanisms without defined trigger

›Autoimmune disease associated: 14%

›SLE and antiphospholipid syndrome predominate

›HIV-associated: 13%

›May require antiretroviral therapy optimization

›Pregnancy-associated: 7%

›Postpartum period also at risk

Pathophysiology

›ADAMTS13 deficiency mechanism

›ADAMTS13 enzyme function

›Cleaves ultra-large von Willebrand factor (UL-vWF) multimers

›Prevents pathologic platelet adhesion to endothelial surface

›Immune inhibition in iTTP

›IgG autoantibodies against ADAMTS13 (inhibitory and non-inhibitory types)

›Inhibitory antibodies block catalytic activity

›Non-inhibitory antibodies accelerate ADAMTS13 clearance

›Consequence of deficiency

›UL-vWF multimers accumulate on endothelial surface

›Platelet binding and activation in microvasculature

›Platelet-rich thrombi occlude capillaries and arterioles

›Organ injury mechanisms

›Microthrombus formation in multiple organs

›Brain: cortical and subcortical microinfarcts

›Heart: coronary microthrombi causing troponin elevation

›Kidney: glomerular capillary occlusion (less severe than aHUS)

›Skin: dermal capillary thrombosis causing petechiae

›Mechanical red cell hemolysis

›RBCs fragment on fibrin strands in microthrombi

›Schistocyte formation

›Hemoglobin release drives LDH elevation

›Thrombocytopenia mechanism

›Platelet consumption in microthrombi

›Not immune-mediated platelet destruction (contrast with ITP)

Therapeutic Considerations

›Triple therapy paradigm

›Standard of care: TPE + corticosteroids + rituximab

›TPE removes inhibitory antibodies and replaces ADAMTS13

›Corticosteroids suppress autoantibody production and reduce inflammation

›Rituximab depletes B cells, eliminating autoantibody source

›Addition of rituximab reduces relapse rate significantly

›Caplacizumab as fourth pillar

›Complementary mechanism: blocks platelet-vWF interaction

›Does not address underlying autoimmune cause

›Reduces organ damage by rapidly reducing platelet consumption

›Must be continued until ADAMTS13 recovers to prevent rebound

›ADAMTS13-guided preemptive therapy

›Monitoring protocol in remission

›ADAMTS13 activity every 1 to 3 months initially

›Then every 3 to 6 months in sustained remission

›Preemptive rituximab threshold

›ADAMTS13 < 10 to 20% without clinical relapse

›OR 0.09 for clinical relapse with preemptive rituximab vs. observation

›Reduces relapse episodes from 0.5 per year to 0 per year

›Congenital TTP management

›No immune mechanism; no rituximab or steroids

›FFP infusion as ADAMTS13 replacement therapy

›Prophylactic FFP every 2 to 3 weeks prevents crises

›Emerging therapy: recombinant ADAMTS13 (investigational)

›Clinical trials underway

›May replace FFP infusions

11Patient Discharge Instructions/pdi37

copy discharge instructions

›Diagnosis and why it is serious

›Thrombotic thrombocytopenic purpura (TTP) is a rare blood condition where small clots form inside blood vessels

›These clots can injure the brain, heart, and kidneys

›You were treated in hospital with plasma exchange to remove harmful antibodies from your blood

›You will need close follow-up with your blood specialist (hematologist)

›Blood tests will be done regularly to make sure the disease stays in remission

›A blood test called ADAMTS13 will be monitored; if it falls, medication may be restarted to prevent relapse

›Medications at home

›Take all prescribed medications exactly as directed

›Do not stop prednisone or other steroids suddenly without medical guidance

›If you have a caplacizumab injection, use it daily as prescribed and keep it refrigerated

›Avoid quinine-containing products (certain tonics, some herbal remedies)

›Even small amounts can re-trigger TTP in sensitive individuals

›Check all beverage and supplement labels

›No aspirin, ibuprofen, or NSAIDs without asking your doctor first

›These medications may increase bleeding risk

›Activity and diet

›Light activity is fine; avoid contact sports or activities with high bleeding risk until told otherwise

›Platelet counts can fluctuate in early remission

›Eat a balanced diet and stay well-hydrated

›No specific dietary restrictions

›Warning signs: return to the emergency room immediately for

›New or worsening headache, confusion, or memory problems

›Vision changes: blurring, double vision, loss of vision

›Weakness or numbness in the face, arm, or leg

›Seizure or loss of consciousness

›Chest pain or palpitations

12References/r29

Guidelines and key sources

›Primary guidelines

›Zheng XL et al. 2025 Focused Update of the 2020 ISTH Guidelines for Management of TTP. J Thromb Haemost. 2025. PMID 40533296

›Covers triple therapy, caplacizumab, ADAMTS13 monitoring, preemptive rituximab

›Zheng XL. The Standard of Care for Immune TTP Today. J Thromb Haemost. 2021. PMID 34060225

›Comprehensive review of TPE, rituximab, and caplacizumab

›Pishko AM, Li A, Cuker A. Immune Thrombotic Thrombocytopenic Purpura. JAMA. 2025

›Primary evidence base for this document; diagnostic criteria, epidemiology, management

Key clinical trials and studies

›Landmark trials and evidence

›HERCULES trial: Caplacizumab vs. placebo in acquired TTP

›Knoebl P et al. J Thromb Haemost. 2020. PMID 32199003

›Demonstrated faster platelet recovery and reduced refractoriness

›PLASMIC score validation: Paydary K et al. Transfusion. 2020. PMID 32757237

›AUROC 0.96 for predicting ADAMTS13 < 10%

›Preemptive rituximab: Hie M et al. Blood. 2014. PMID 24869941

›OR 0.09 for clinical relapse with preemptive rituximab

›Cardiac troponin prognosis: Benhamou Y et al. J Thromb Haemost. 2015. PMID 25403270

›cTnI elevation predicts death and refractoriness

›Rapid ADAMTS13 assays: Deshpande SR et al. Blood. 2025. PMID 40258188

›Meta-analysis supporting rapid assay use for early diagnosis

›Long-term relapse risk: Doyle AJ et al. Blood. 2023. PMID 36322971

›40% relapse at 5 years; role of anti-CD20 therapy

Coding and classification

›ICD-10 and classification

›ICD-10 M31.1: Thrombotic microangiopathy (TTP)

›Applies to both immune and congenital TTP

›Additional codes for associated conditions as appropriate

Learn More: Thrombotic Thrombocytopenic Purpura (TTP)→

Comprehensive Clinical Review

Structured, evidence-informed, long-form analysis for deeper understanding.

Evidence & Review

Reviewed by SymptomDx Medical Team · Last reviewed August 18, 2026

SymptomDx is an educational tool for medical professionals. It does not replace clinical judgment. Verify all clinical data and drug dosages with authoritative sources.