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HEM 302 > Haemostasis II: Vascular and Platelet Disorders > Flashcards

Haemostasis II: Vascular and Platelet Disorders Flashcards

1
Q

What are the two types of vascular purpuras?

A

Acquired, congenital/inherited

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2
Q

What are the acquired vascular disorders?

A

Acquired vascular purpuras include simple purpura, senile purpura, symptomatic purpuras, Henoch-Schonlein syndrome, orthostatic purpura, mechanical purpura, steroid purpura, and psychogenic purpura.

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3
Q

What infections can cause symptomatic purpuras?

A

Infections causing symptomatic purpuras include typhoid, subacute bacterial endocarditis, meningococcal meningitis, and gram-negative septicemia.

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4
Q

What drugs and chemicals can cause symptomatic purpuras?

A

Drugs and chemicals causing symptomatic purpuras include penicillin, chlorothiazides, salicylates, streptomycin, sulfonamides, phenacetin, amidopyrine, phenylbutazone, gold, arsenic, bismuth, and mercury.

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5
Q

What are some systemic conditions that can cause symptomatic purpuras?

A

Systemic conditions causing symptomatic purpuras include uraemia, Cushing’s syndrome, corticosteroid therapy, scurvy, dysproteinemias, cryoglobulinemia, macroglobulinemia, and myeloma.

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6
Q

What are congenital vascular purpuras?

A

Congenital vascular purpuras include hereditary hemorrhagic telangiectasia and purpura associated with congenital connective tissue diseases such as Ehlers-Danlos syndrome, Marfan syndrome, and osteogenesis imperfecta.

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7
Q

What is hereditary hemorrhagic telangiectasia also known as?

A

Hereditary hemorrhagic telangiectasia is also known as Osler-Weber-Rendu disease.

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8
Q

What conditions are associated with congenital connective tissue disease causing vascular purpuras?

A

Conditions associated with congenital connective tissue diseases causing vascular purpuras include Ehlers-Danlos syndrome, Marfan syndrome, and osteogenesis imperfecta.

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9
Q

What are the primary manifestations of platelet disorders?

A

Primary manifestations of platelet disorders include petechiae, purpura, ecchymoses, epistaxis, gingival hemorrhage, and menorrhagia.

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10
Q

What types of abnormalities can platelet disorders include?

A

Platelet disorders can include both quantitative and qualitative abnormalities.

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11
Q

What are hereditary qualitative platelet abnormalities?

A

Hereditary qualitative platelet abnormalities can affect glycoprotein adhesion receptors, platelet granules, platelet coagulation activity, and signal transduction and secretion.

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12
Q

What is Glanzmann thrombasthenia?

A

Glanzmann thrombasthenia is an inherited hemorrhagic disorder characterized by severely reduced or absent platelet aggregation due to qualitative or quantitative glycoprotein abnormalities.

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13
Q

What are the lab features of Glanzmann thrombasthenia?

A

Lab features of Glanzmann thrombasthenia include normal platelet count, markedly prolonged bleeding time, and poor platelet aggregation using agonists like ADP, epinephrine, collagen, or ristocetin.

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14
Q

What are acquired qualitative platelet disorders?

A

Acquired qualitative platelet disorders are frequent causes of abnormal platelet functions, bleeding diathesis, and prolonged bleeding time.

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15
Q

What are common causes of acquired qualitative platelet disorders?

A

Common causes of acquired qualitative platelet disorders include drugs, hematologic diseases, and other systemic disorders.

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16
Q

Which drugs are frequent causes of platelet dysfunction?

A

Drugs frequent causes of platelet dysfunction include NSAIDs (aspirin, indomethacin, ibuprofen, naproxen, piroxicam), antibiotics (penicillins, cephalosporins), anticoagulants (heparin), fibrinolytics (streptokinase, tPA, urokinase), antifibrinolytics (ε-aminocaproic acid), cardiovascular drugs (propranolol, nifedipine, verapamil), psychotropic drugs (imipramine, amitriptyline, chlorpromazine, promethazine, haloperidol), anesthetics (procaine, cocaine, halothane), and others (mithramycin, daunorubicin, BCNU, ethanol, chlorpheniramine).

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17
Q

Which haematologic diseases can cause acquired qualitative platelet disorders?

A

Haematologic diseases causing acquired qualitative platelet disorders include chronic myeloproliferative disorders (essential thrombocythemia, polycythemia vera, myelofibrosis), leukemias (AML, ALL, HCL, MDS), and dysproteinemias (IgA myeloma, Waldenström’s macroglobulinemia).

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18
Q

What systemic disorders are associated with abnormal platelet function?

A

Systemic disorders associated with abnormal platelet function include uraemia, antiplatelet antibodies (ITP, SLE, platelet alloimmunization), cardiopulmonary bypass, chronic liver disease, DIC, atopic asthma, hay fever, and Wilms tumor.

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19
Q

What are the two main categories of thrombocytopenia?

A

The two main categories of thrombocytopenia are congenital and acquired.

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20
Q

What are some congenital causes of thrombocytopenia?

A

Congenital causes of thrombocytopenia include congenital amegakaryocytic thrombocytopenia, TAR syndrome, and X-linked thrombocytopenia.

21
Q

What are causes of bone marrow suppression leading to thrombocytopenia?

A

Causes of bone marrow suppression leading to thrombocytopenia include bone marrow infiltration, infections (parvovirus, HBV, HIV, CMV), chemotherapy, radiotherapy, aplastic anemia, B12 and folate deficiency, paroxysmal nocturnal hemoglobinuria (PNH), myelodysplastic syndromes (MDS), and acute leukemias.

22
Q

What are causes of accelerated platelet destruction in thrombocytopenia?

A

Causes of accelerated platelet destruction in thrombocytopenia include autoimmune mediated (ITP), secondary immune mediated (lymphoproliferative diseases, infections, pregnancy-related HELLP syndrome, collagen-vascular diseases), alloimmune causes (post-transfusion purpura, neonatal thrombocytopenia), non-immune (TTP, HUS, DIC), hypersplenism, and drug-induced (heparin-induced thrombocytopenia, aldomet, penicillin).

23
Q

What is immune thrombocytopenic purpura (ITP)?

A

Immune thrombocytopenic purpura (ITP) is a common acquired autoimmune platelet disorder characterized by accelerated platelet destruction by antiplatelet antibodies and increased marrow production of platelets.

24
Q

What are some clinical features of ITP?

A

Clinical features of ITP include epistaxis, petechiae, ecchymoses, purpura, menorrhagia, hematuria, hemoptysis, gastrointestinal bleeding, and non-palpable purpura that do not blanch on pressure.

25
Q

What lab tests are used for diagnosing ITP?

A

Lab tests for diagnosing ITP include FBC showing thrombocytopenia, platelet anisocytosis, increased PDW and MPV, possibly normal Hb, and absence of poikilocytes and schistocytes; bleeding time correlates inversely with platelet count; inconsistent leucocytosis or leucopenia; and bone marrow aspiration or trephine biopsy showing normal or increased megakaryocytes.

26
Q

What is the first line of treatment for ITP?

A

First line of treatment for ITP includes glucocorticoids such as oral prednisone 1-2 mg/kg/day or high-dose methylprednisolone IV.

27
Q

What are the side effects of glucocorticoids used in ITP treatment?

A

Side effects of glucocorticoids in ITP treatment include facial puffiness, weight gain, folliculitis, hyperglycemia, hypertension, cataract, osteoporosis, opportunistic infection, and behavioral changes.

28
Q

What is a surgical treatment option for ITP?

A

Surgical treatment option for ITP is splenectomy, indicated in patients with persistently low platelet counts (<10 x 10^9/L).

29
Q

What post-surgical considerations are there for splenectomy in ITP patients?

A

Post-surgical considerations for splenectomy in ITP patients include pneumococcal vaccination (Pneumovax) and prophylactic Pen-V 250mg BD orally.

30
Q

What is the purpose of Pneumovax in ITP treatment?

A

Pneumovax is used in ITP treatment to prevent infections post-splenectomy.

31
Q

What might be indicated if a patient remains refractory after splenectomy in ITP treatment?

A

If a patient remains refractory after splenectomy in ITP treatment, it may indicate the presence of an accessory spleen.

32
Q

Which vascular purpura is associated with aging?

A

Senile purpura is associated with aging.

33
Q

What is the treatment approach for asymptomatic ITP patients with platelet counts over 50 x 10^9/L?

A

The treatment approach for asymptomatic ITP patients with platelet counts over 50 x 10^9/L is observation.

34
Q

What emergency treatments are used for ITP patients with platelet counts below 10 x 10^9/L?

A

Emergency treatments for ITP patients with platelet counts below 10 x 10^9/L include glucocorticoids, IVIg, and possibly splenectomy.

35
Q

What is the significance of haemorrhagic bullae in ITP patients?

A

Haemorrhagic bullae in ITP patients signify severe bleeding and warrant immediate therapy.

36
Q

How does uraemia affect platelet function?

A

Uraemia affects platelet function by causing acquired qualitative platelet disorders.

37
Q

What is the role of IVIg in ITP treatment?

A

The role of IVIg in ITP treatment is to rapidly increase platelet counts in emergency situations.

38
Q

What is a common cause of drug-induced thrombocytopenia?

A

A common cause of drug-induced thrombocytopenia is heparin-induced thrombocytopenia (HIT).

39
Q

What are platelet granules abnormalities in hereditary qualitative platelet disorders?

A

Platelet granules abnormalities in hereditary qualitative platelet disorders include gray platelet syndrome, Quebec platelet disorder, and δ-storage pool deficiency.

40
Q

Which platelet disorder is characterized by abnormalities in glycoprotein adhesion receptors?

A

Glanzmann thrombasthenia is characterized by abnormalities in glycoprotein adhesion receptors.

41
Q

What is a common treatment for platelet alloimmunization causing platelet dysfunction?

A

A common treatment for platelet alloimmunization causing platelet dysfunction is IVIg.

42
Q

What are examples of NSAIDs that cause platelet dysfunction?

A

Examples of NSAIDs that cause platelet dysfunction include aspirin, indomethacin, ibuprofen, naproxen, and piroxicam.

43
Q

What are examples of antibiotics that cause platelet dysfunction?

A

Examples of antibiotics that cause platelet dysfunction include penicillins (ampicillin, carbenicillin, penicillin G), cephalosporins (cephalothin, cefoxitin, cefotaxime), miconazole, and nitrofurantoin.

44
Q

What are examples of cardiovascular drugs that cause platelet dysfunction?

A

Examples of cardiovascular drugs that cause platelet dysfunction include propranolol, nifedipine, and verapamil.

45
Q

What are examples of psychotropic drugs that cause platelet dysfunction?

A

Examples of psychotropic drugs that cause platelet dysfunction include imipramine, amitriptyline, chlorpromazine, promethazine, and haloperidol.

46
Q

What are examples of anaesthetics that cause platelet dysfunction?

A

Examples of anaesthetics that cause platelet dysfunction include procaine, cocaine, and halothane.

47
Q

What systemic disorder is associated with antiplatelet antibodies causing platelet dysfunction?

A

A systemic disorder associated with antiplatelet antibodies causing platelet dysfunction is systemic lupus erythematosus (SLE).

48
Q

What is a common haematologic disease causing acquired qualitative platelet disorders?

A

A common haematologic disease causing acquired qualitative platelet disorders is chronic myeloproliferative disorder.

HEM 302 (5 decks)

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