Phys · haematological
Bleeding Disorders AND Thrombocytopenia
Also known as Bleeding Disorders AND Thrombocytopenia · immune thrombocytopenia · ITP · thrombotic thrombocytopenic purpura · TTP · disseminated intravascular coagulation · DIC · heparin-induced thrombocytopenia · HIT · von Willebrand disease · hemophilia
Consultant-physician depth guide to bleeding disorders and thrombocytopenia — ITP (ASH 2019, international consensus 2019), TTP and the PLASMIC score, ADAMTS13, caplacizumab, DIC (ISTH 2025 update), atypical HUS and complement, von Willebrand disease, hemophilia A with emicizumab, heparin-induced thrombocytopenia, and the tourniquet test that distinguishes platelet versus coagulation defects. Structured for FRACP DWE/DCE, MRCP and ABIM preparation.
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- TTP is a haematological emergency — microangiopathic haemolytic anaemia plus thrombocytopenia plus ADAMTS13 < 10 percent demands urgent plasma exchange within hours, not days, to prevent death from cerebral or myocardial microvascular thrombosis
- Severe thrombocytopenia (platelet < 10 × 10⁹/L) plus mucocutaneous bleeding or visceral bleed — IVIG 1 g/kg/day for 2 days plus high-dose corticosteroid
- Heparin-induced thrombocytopenia (HIT) paradoxically thromboses — fall in platelet ≥ 50 percent within 5 to 14 days of heparin exposure mandates immediate cessation of all heparin and switch to argatroban or danaparoid
- Disseminated intravascular coagulation in sepsis or malignancy — PT/APTT prolonged, fibrinogen low, D-dimer markedly elevated, platelets falling; treat the cause plus supportive blood product support
- Acquired haemophilia with inhibitor — sudden life-threatening bleed in an elderly patient with normal platelets but isolated prolonged APTT that does not correct on mixing; urgent factor VIII inhibitor assay and immunosuppression
- Neonatal alloimmune thrombocytopenia (NAIT) — severe thrombocytopenia in an otherwise healthy neonate; intracranial haemorrhage risk warrants urgent matched platelet transfusion
Bleeding Disorders AND Thrombocytopenia
The answer first
The first decision in any bleeding or thrombocytopenic patient is to localise the defect by clinical pattern and basic coagulation: platelet-type bleeding (petechiae, epistaxis, menorrhagia, mucosal oozing) versus coagulation-type bleeding (deep haematomas, haemarthroses, delayed rebleeding) versus mixed. [1]
A physician-level answer rests on four pillars: [1]
- Recognise the emergency. TTP is a true medical emergency: microangiopathic haemolytic anaemia (MAHA) plus thrombocytopenia, ADAMTS13 < 10 percent. Plasma exchange must begin within hours. DIC in sepsis demands aggressive treatment of the trigger plus blood product support. Acquired haemophilia with isolated prolonged APTT and normal platelets presents with catastrophic bleeding in the elderly.
- Distinguish isolated thrombocytopenia from pancytopenia. Isolated thrombocytopenia with normal haemoglobin and white cells is usually ITP, drug-induced, gestational, HIV-related, or a thrombotic microangiopathy. Pancytopenia points to marrow causes — aplastic anaemia, leukaemia, myelodysplasia, megaloblastic, infiltration.
- Define the mechanism before treatment. Autoimmune (ITP) needs immunosuppression; consumptive (DIC, TTP, HUS) needs cause-directed therapy; underproduction (marrow failure) needs haemopoietic support; sequestration (hypersplenism) needs the underlying cause treated.
- Stratify bleeding risk by platelet count and clinical context. Spontaneous bleeding rarely occurs above 20 × 10⁹/L unless there is platelet dysfunction or fever. Procedural thresholds: dental extraction ≥ 30 × 10⁹/L; major surgery ≥ 50 × 10⁹/L; neurosurgery/eye ≥ 100 × 10⁹/L. [1]
DWE high-yield: The single most discriminating question in acute thrombocytopenia is "is there MAHA?" — schistocytes on the film, elevated LDH, low haptoglobin, indirect bilirubin. If yes, the differential narrows immediately to TTP, atypical HUS, DIC, malignant hypertension, HELLP, or haematologic malignancy. Plasma exchange is started empirically if TTP cannot be excluded within hours — wait for ADAMTS13 only if it does not delay exchange. [3] [4]
References12ShowHide
- [1]Provan D, Arnold DM, Bussel JB, et al. Updated international consensus report on the investigation and management of primary immune thrombocytopenia. Blood Adv, 2019.PMID 31770441
- [2]Neunert C, Terrell DR, Arnold DM, et al. American Society of Hematology 2019 guidelines for immune thrombocytopenia. Blood Adv, 2019.PMID 31794604
- [3]Zheng XL, Vesely SK, Cataland SR, et al. ISTH guidelines for the diagnosis of thrombotic thrombocytopenic purpura. J Thromb Haemost, 2020.PMID 32914582
- [4]Bendapudi PK, Hurwitz S, Fry A, et al. Derivation and external validation of the PLASMIC score for rapid assessment of adults with thrombotic microangiopathies: a cohort study. Lancet Haematol, 2017.PMID 28259520
- [5]Ghanima W, Cooper N, Rodeghiero F, et al. Thrombopoietin receptor agonists: ten years later. Haematologica, 2019.PMID 31073079
- [6]Connell NT, Flood VH, Brignardello-Petersen R, et al. ASH ISTH NHF WFH 2021 guidelines on the management of von Willebrand disease. Blood Adv, 2021.PMID 33570647
- [7]Oldenburg J, Mahlangu JN, Kim B, et al. Emicizumab Prophylaxis in Hemophilia A with Inhibitors. N Engl J Med, 2017.PMID 28691557
- [8]Iba T, Levy JH, Levi M, et al. Updated definition and scoring of disseminated intravascular coagulation in 2025: communication from the ISTH SSC. J Thromb Haemost, 2025.PMID 40216223
- [9]Brocklebank V, Walsh PR, Smith-Jackson K, et al. Atypical hemolytic uremic syndrome in the era of terminal complement inhibition: an observational cohort study. Blood, 2023.PMID 37369098
- [10]Joly BS, Coppo P, Veyradier A Thrombotic thrombocytopenic purpura. Blood, 2017.PMID 28416507
- [11]Dasararaju R, Singh N, Mehta A Heparin induced thrombocytopenia: review. Expert Rev Hematol, 2013.PMID 23991928
- [12]Vianelli N, Auteri G, Buccisano F, et al. Refractory primary immune thrombocytopenia (ITP): current clinical challenges and therapeutic strategies. Ann Hematol, 2022.PMID 35201417