Page 520 - Clinical Hematology_ Theory _ Procedures ( PDFDrive )

Principles of Blood Collection and
26 Disorders of Primary Hemostasis and

CHAPTER Trombosis Vasculature and Platelets
Processing

KEY TERMS

autoim m une hem olytic anem ia im m une throm bocytopenia (ITP) purpura

ecchym osis im m une throm bocytopenic sarcoidosis
essential athrom bia purpura system ic lupus erythem atosus

brinolysis neonatal autoim m une (SLE)
Glanzm ann’s throm basthenia throm bocytopenia throm bocytopenia

hem atom a petechiae throm bocytosis

LEARNING OUTCOMES

Vascular abnormalities Compare the hereditary defects of platelet function (with thrombo-

■ De ne the terms petechiae, ecchymoses, hematoma. cytopenia): Wiskott-Aldrich syndrome, MYH9-related thrombocyto-

■ De ne the term purpura and describe various vascular conditions penia syndromes (May-Hegglin anomaly).
that can produce this condition. List and summarize the characteristics of primary (malignant) versus

■ Describe the effect of mechanical force on blood vessels. secondary (reactive) thrombocytosis, including examples of disor-
Explain the genetic basis of hereditary hemorrhagic telangiectasia ders within each category.

(HHT). Qualitative characteristics of platelets:

Compare the vasculitis disorders of antineutrophil cytoplas- thrombocytopathy/ thrombocythemia

mic antibody–positive vasculitis, cryoglobulinemia, hyper-

gammaglobulinemic purpura, and Henoch-Schönlein purpura Compare types of acquired platelet dysfunction and include hemo-

(HSP). lytic-uremic syndrome and HELLP syndrome.

Describe the role of the vasculature in disseminated intravascular ■ Compare categories of platelet dysfunctions, including examples of

coagulation (DIC). disorders within each category.

Compare the etiology, pathophysiology, and laboratory testing for ■ Compare hereditary platelet adhesion and aggregation disorders:

bleeding disorders of perivascular tissue: Ehlers-Danlos syndromes, Bernard-Soulier syndrome, Glanzmann’s thrombasthenia, storage

pseudoxanthoma elasticum, scurvy, steroid-induced purpura, and granule abnormalities, and von Willebrand’s disease.

solar purpura (senile purpura). ■ Discuss secondary aggregation disorders: hereditary storage pool

Describe purpura associated with infections and miscellaneous defect and hereditary aspirin-like defects.

causes. Case study

Analyze the patient history, clinical signs and symptoms, and labo-
Quantitative platelet disorders ratory data for the stated case study, answer the related critical

■ De ne thrombocytopenia, thrombocytosis, and thrombocythemia. thinking questions, and decide the most likely diagnosis.

■ De ne the terms thrombocytopenia and thrombocytosis.

■ Cite at least two symptoms of thrombocytopenia. NOTE:

Compare the characteristics of disorders of platelet production, dis- indicates MLT and MLS core content

orders of platelet destruction or utilization, and disorders of platelet ■
indicates MLT (optional) and MLS advanced content
distribution and dilution.

VASCULAR ABNORMALI IES the icrocircu ation ex resses itse as the con ition o
purpura, which is characterize by obvious he orrhages

Disor ers o the icrocircu ation, ate ets, or as a into the skin an ucous e branes that a ear as exten-

roteins ay cause abnor a b ee ing. Abnor a b ee - sive areas o re or ark- ur e isco oration. I s a , ur-

ing invo ving the oss o re b oo ce s (RBCs) ro ish he orrhagic s ots on the skin or ucous e branes

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