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Pulmonary embolism · Chapter 5 of 8

Every test, and when it is worth doing

The tests everyone should have, the ones the review should include, every thrombophilia and genetic test with what it means, and the ones that are sold but useless.

People who have had a clot are often told that "all the tests were done" or that "there's a genetic test for that", and neither is usually true in the way they imagine. This chapter is the full list: the tests everyone should have, the ones the three-month review should include, the thrombophilia and genetic tests with what each one means, the extended tests for unusual clots, and the tests that are sold but not worth doing. It applies to a DVT and a pulmonary embolism alike; the blood clots topic links here rather than repeating it.

The one rule. A test is worth doing when its result would change something: whether treatment stops, which drug is used, what a relative is advised, or how a pregnancy or an operation is managed. A test done in the first week, on an anticoagulant, "to see what's there", will usually be wrong, will need repeating, and changes nothing. Both the British Society for Haematology and its American counterpart say so in as many words.

1. Tier one: at diagnosis, everyone

TestWhyWhat an abnormal result can mean
Full blood countBaseline before anticoagulation (NG158 1.3.4)Low platelets: bleeding risk, or antiphospholipid syndrome, or heparin-induced thrombocytopenia if on heparin. Very high haemoglobin or platelets: a possible myeloproliferative neoplasm (tier four). Anaemia: bleeding, or cancer
Kidney function (creatinine, eGFR, and creatinine clearance calculated from weight)Chooses the anticoagulant and its doseClearance under 30 restricts most DOACs; under 15 excludes them. Protein in the urine with low albumin points to nephrotic syndrome, which loses antithrombin and causes clots
Liver functionBaseline; drug choiceSevere liver disease restricts DOACs and lowers the clotting proteins measured in tier three; a clot in the liver veins needs its own workup
Clotting screen (PT or INR, APTT)Baseline before the first doseA prolonged APTT before any anticoagulant, in someone who clots rather than bleeds, is the classic clue to a lupus anticoagulant
D-dimerDiagnosis only, and only in the "unlikely" arm of the pathwayHas no role after the diagnosis is made; a raised level later does not mean the clot is back
Troponin, BNP, lactate, blood gasSeverity of a pulmonary embolism, not the diagnosisSee the hospital checklist
GlucoseRoutineUndiagnosed diabetes is common and changes the long-term picture
Pregnancy testAnyone who could be pregnantChanges the drug (heparin, not tablets), the imaging and the follow-up
UrinalysisCheap and often omittedBlood: kidney or bladder cancer to exclude. Protein: nephrotic syndrome
Calcium, CRP or ESRNot required by NG158; commonly addedA high calcium or a high inflammatory marker with no infection is a cancer or inflammatory-disease clue and prompts examination, not a scan

None of these results is waited for before the first dose. They are reviewed within 24 hours and the drug adjusted if they demand it.

2. Tier two: the three-month review, after an unprovoked clot

The purpose of this tier is cancer, and the evidence is that a careful clinical assessment finds what a body scan finds (see the after-hospital chapter). NICE's list (1.8.1): the history, a physical examination, and a review of the tier-one bloods. In practice that should mean:

  • Weight and any weight loss; appetite; night sweats; a change in bowel habit; a cough that has not gone; a new lump; unexplained bleeding from anywhere.
  • Examination of the chest, abdomen, lymph nodes, breasts, testes or prostate, and skin.
  • Repeat full blood count, liver function, kidney function; urinalysis; calcium if not done.
  • Screening programmes brought up to date for the person's age: bowel (FIT test, 50 or 54 to 74 depending on nation), breast (50–70), cervical (25–64), abdominal aortic aneurysm (men at 65), and lung screening for current and former heavy smokers aged 55–74 where the programme has reached their area.
  • Not routinely: CT of the chest, abdomen and pelvis, tumour markers (PSA, CA-125, CEA, CA19-9), endoscopy, or mammography outside the programme. Each generates false alarms and none finds more cancer than the examination does. Any of them is done when a symptom or sign points to it, through the suspected-cancer pathway.

3. Tier three: thrombophilia, and what each test means

Ordered only when the result would change the plan (NG158 1.9): after an unprovoked clot when stopping anticoagulation is being considered, or where a family pattern makes a high-risk inherited deficiency plausible. Not after a provoked clot; not in someone who will continue anticoagulation regardless; not routinely in relatives. When it is ordered, this is what is on the form.

TestWhat it detectsHow common; how much it raises riskWhat a positive result changes
Lupus anticoagulant (a clotting-based test, two methods)Antiphospholipid syndrome: an acquired autoimmune tendency to clot in veins and arteries, and to lose pregnanciesAround 1 in 10 people with an unprovoked clot has some antiphospholipid antibody; persistent triple positivity is much rarer and carries the highest recurrence risk of any thrombophiliaContinue anticoagulation, and if triple-positive use warfarin rather than a DOAC (the TRAPS trial, and NG158 1.3.20). A positive result must be repeated at least 12 weeks later to count. Changes pregnancy management completely
Anticardiolipin antibodies (IgG and IgM)—
Anti-β2-glycoprotein I antibodies (IgG and IgM)—
Factor V Leiden (a genetic test; some laboratories screen first with an "activated protein C resistance" clotting test)A variant that makes factor V resistant to being switched offThe commonest inherited thrombophilia: about 1 in 20 people of European ancestry carry one copy, which raises first-clot risk roughly 3–5 fold; two copies (rare) raise it far more. Its effect on recurrence is modestRarely the duration of treatment on its own. Firms up advice never to use combined oestrogen, prophylaxis in pregnancy and after surgery, and counselling for relatives
Prothrombin G20210A (genetic)A variant that raises prothrombin levelsAbout 1 in 50 of European ancestry; raises first-clot risk 2–3 fold; modest effect on recurrenceAs for factor V Leiden. Carrying both variants is more significant
Antithrombin activityDeficiency of the protein that heparin works throughRare, about 1 in 2,000–5,000; the highest-risk inherited deficiency, raising clot risk 10–20 fold, with clots often before 40Usually long-term anticoagulation; heparin may work less well ("heparin resistance"); antithrombin concentrate around surgery and childbirth; selective testing of first-degree relatives is reasonable (BSH 2022)
Protein C activityDeficiency of a natural anticoagulantAbout 1 in 300–500; raises risk several-foldOften long-term anticoagulation; starting warfarin needs heparin cover to avoid skin necrosis; selective relative testing
Protein S (free antigen, and activity)Deficiency of protein C's cofactorSimilar to protein C; the hardest of the three to measure reliablyAs for protein C
Monogenic thrombophilia gene panel (Genomics England)Sequencing of the antithrombin, protein C, protein S, factor V, prothrombin and fibrinogen genes, among othersFor clots under 40 that were spontaneous or weakly provoked and are also present in a first-degree relative, requested by a haematologist or geneticistConfirms a deficiency found on activity testing, explains a strong family history, and allows cascade testing of relatives who want it

The timing rules, which is where most of these tests go wrong. Antithrombin is lowered by heparin and by the clot itself. Protein C and protein S are lowered by warfarin, the acute clot, liver disease, pregnancy and oestrogen. The lupus anticoagulant test gives false results on heparin, warfarin and especially DOACs, and the clotting-based screen for factor V Leiden is unreliable on DOACs too. So: the clotting-based tests are done four to six weeks after anticoagulation stops, or with the laboratory's advice on neutralising the drug in the sample; the genetic tests can be done at any time; and a low protein C, protein S or antithrombin found in hospital is repeated before anyone is told they have a deficiency. Positive antiphospholipid tests are repeated at twelve weeks before the diagnosis is made.

4. Tier four: extended tests for unusual, young or recurrent clots

Justified by a clot in an unusual site (arm, abdomen, brain veins), clots at a young age, clots in both arteries and veins, a clot despite anticoagulation, an abnormal blood count, or features of another disease. Ordered through a haematologist.

TestLooks forWhenWhat it changes
JAK2 V617F mutation (then CALR and MPL if negative)A myeloproliferative neoplasm, which can present with a clot before the blood count is abnormalAny abdominal or brain-vein clot; any clot with a raised haemoglobin or platelet count; recurrent unexplained clotsA haematology diagnosis with its own treatment; usually long-term anticoagulation
PNH clone (flow cytometry for CD55/CD59 or FLAER)Paroxysmal nocturnal haemoglobinuria, a rare acquired condition that clots in unusual veinsAbdominal or brain-vein clots; clots with haemolysis or a low blood countTreatable with a specific antibody drug
Autoimmune screen (ANA, anti-dsDNA, complement, ANCA; ESR)Lupus and other connective tissue disease, vasculitis, Behçet'sAntiphospholipid antibodies positive, or symptoms: rash, joint pain, mouth ulcers, eye inflammation, feverRheumatology referral; immunosuppression treats the cause
Urine protein-to-creatinine ratio and serum albuminNephrotic syndrome (loses antithrombin in the urine)Protein on the dipstick; swollen anklesRenal referral; the clot risk falls when the kidney disease is treated
Myeloma screen (serum protein electrophoresis, free light chains)Myeloma and related conditions, which thicken the bloodOlder people with unexplained clots, bone pain, anaemia or high calciumHaematology diagnosis
Heparin-induced thrombocytopenia antibodies (anti-PF4)An immune reaction to heparin that causes clots and a falling platelet countPlatelets fall by half between day 5 and 10 of heparin, or a new clot on heparinStop all heparin; use argatroban or fondaparinux; never heparin again
HomocysteineHomocystinuria, a rare inherited metabolic diseaseOnly with the features: clots young, tall thin build, dislocated lenses, learning difficulty. Not as a routine "risk factor" test: lowering a mildly raised level with vitamins does not prevent clotsMetabolic referral; a specific diet and vitamin treatment
Factor VIII levelPersistently high levels associate with clotsSometimes measured in unexplained recurrenceNothing specific; it is a research marker more than a clinical one
Fibrinogen and thrombin timeDysfibrinogenaemia, a rare structural defectUnexplained recurrence with a family history and odd clotting-screen resultsHaematology-led
Cancer investigation directed by a findingThe cancer the examination or bloods pointed toAny symptom or sign, a high calcium, blood in the urine, anaemiaThe suspected-cancer pathway for that site

5. Tests that are sold but not worth doing

  • MTHFR (C677T, A1298C): a common variant with no clinically significant link to clots. The BSH guideline states outright that it should not be tested for; nor should PAI-1 (SERPINE1) variants.
  • "Full thrombophilia screens" ordered on the ward in the first week or on an anticoagulant: half the results will be uninterpretable and need repeating.
  • Direct-to-consumer genetic panels that report factor V Leiden or prothrombin status alongside dozens of unvalidated variants: the two real ones are worth knowing only in the situations above, and the rest are noise.
  • Homocysteine as a routine risk marker, for the reason above.
  • Repeated D-dimers after diagnosis to "check the clot is gone" or to decide on stopping treatment: NICE says not to use D-dimer for that decision.
  • Tumour markers and whole-body scans as cancer screening after a clot (tier two).

6. For your relatives

Routine testing of family members is not recommended (NG158 1.9.5; BSH 2022). The exception is a first-degree relative of someone with a confirmed protein C, protein S or antithrombin deficiency, where the result can change decisions about the combined pill, pregnancy and surgery; testing is offered selectively and with counselling. What a relative of anyone who has had a clot should do regardless of testing: mention the family history at every admission, operation and pregnancy; avoid combined oestrogen where an alternative exists; and know the symptoms.

7. The list, in one paragraph, for the review appointment

"Were the baseline bloods normal, including the blood count, kidney, liver and clotting?" "Has a physical examination been done and is my screening up to date?" If stopping anticoagulation is being discussed after an unprovoked clot: "Should antiphospholipid antibodies be tested, and if a relative has had a clot, should the inherited tests be done, and when, given my treatment?" If the clot was in an unusual place or you are under 40: "Have JAK2 and PNH been tested, and have I been referred to haematology?" And if anyone offers MTHFR or a full panel on the ward: "What would that result change?"

8. Where UK and US guidance differ

The American Society of Hematology's 2023 guideline and the BSH's 2022 guideline reach the same conclusions: test when it changes management, not routinely; nothing for MTHFR or PAI-1; mind the timing. The US guideline is slightly more willing to suggest testing after a clot provoked by hormones or a minor trigger, where a positive result would tip a borderline stopping decision, and US laboratories run wider panels by default. NICE's rules are the narrowest of the three and are the ones that apply on the NHS.