Page 522 - Clinical Hematology_ Theory _ Procedures ( PDFDrive )

506 PART 7 ■ Principles and Disorders of Hemostasis and Thrombosis

b oo vesse s. T ese structures are articu ar y ragi e, an Hypergam m aglobulinem ic Purpura

ur ura can resu t ro very inor trau a. Tis or o ur ura is characterize by o yc ona hy er-

Moya oya isease, a chronic cerebra vascu o athy. T is ga ag obu ine ia associate with recurrent attacks o

isor er can ro uce cerebra in arcts in chi ren an intra- ur ura. T ere are ri ary an secon ary or s. Secon ary

crania he orrhage in a u ts. HP eve o s in atients with un er ying autoi une is-

Cerebra s a vesse isease invo ves the ragi ity o arte- or ers such as Sjögren’s syn ro e an SLE but has been

rio es, usua y en arteries. T is isease is characterize by re orte in atients with chronic he atitis C.

recurrent ische ic strokes, inistrokes, with rogressive Attacks occur a er ro onge stan ing, ong wa ks, exer-

cognitive i air ent. cise, a coho ingestion, or wearing tight- tting c othes.

Vasculitis Henoch-Schönlein Purpura (HSP)
HSP, ur ura rheu atica, or Schön ein-Henoch ur ura.
Vascu itis eans inf a ation o the b oo vesse s, arter- Tis vascu ar isor er occurs ri ari y in chi ren with a

ies, veins, or ca i aries. When such inf a ation occurs, it eak inci ence occurring between 4 an 11 years o age.

causes changes in the wa s o b oo vesse s, such as weaken- Re ish- ur e s ots, which ook ike bruises, are the ost

ing an narrowing that can rogress to the oint o b oo istinctive an universa sign o HSP.

vesse b ockage. HSP is an acute i unog obu in A (IgA)- e iate is-

Exa es o isor ers in this c assi cation inc u e anti- or er characterize by a genera ize vascu itis invo ving the

neutro hi cyto as ic antibo y— ositive vascu itis, cryo- s a vesse s o the skin, the gastrointestina (GI) tract, the

g obu ine ia, hy erga ag obu ine ic ur ura, an ki neys, the joints, an , rare y, the ungs an the centra ner-

Henoch-Schön ein ur ura (HSP). vous syste (CNS).

A bio sy o the skin, an ess co on y ki neys, can be
Antineutrophil Cytoplasm ic Antibody–Positive use to e onstrate vascu itis. S ecia staining techniques

Vasculitis (ANCA) ( irect i unof uorescence) o the bio sy s eci en can be

Antineutro hi cyto as ic antibo ies (ANCAs) are ivi e use with icrosco ic exa ination to ocu ent antibo y

into antineutro hi cyto as ic antibo y (c-ANCA) or anti- e osits o IgA in the wa s o the arterio es o invo ve tissue.

bo y ro ucing a erinuc ear staining o ethano - xe neu-

tro hi s ( -ANCA). ANCA-associate s a vesse vascu itis Vascular Obstruction

is observe in isor ers such as systemic lupus erythematosus Mu ti e b ee ing isor ers are associate with ur ura

(SLE) an Wegener’s granu o atosis. associate with a thro bus, e bo us, or other occ usions

T e hu an ANCAs, escribe or the rst ti e in 1982,

are irecte against antigenic co onents ain y resent in cause by various actors ( ab e 26.1).

ri ary granu es o neutro hi s. ANCAs are sero ogic ark-

ers o ri ary necrotizing syste ic vascu itis, articu ar y in Examples of Bleeding Disorders

Wegener’s granu o atosis. In a ition, these antibo ies have TABLE 26.1 Associated w ith Vascular

a rognostic interest because, in ost cases, their titer is cor- Obstruction

re ate with c inica activity uring the isease.
Vascular Vascular

Cryoglobulinem ia Obstruction Obstruction

Category Component Selected Examples
Cryog obu ine ia vascu itis is associate with cryog obu-

in i une e osits. T e cryog obu ins can reci itate in Thrombus Blood clot Disseminated intravas-

er a vesse s. Wa enströ ’s ri ary acrog obu ine ia cular coagulation

(WM) characterizes this isor er. Ninety ercent o atients Emboli Septic emboli Endocarditis

with WM have MYD88 L265P utations.

Sy to s inc u e cutaneous in arcts an etechiae. Thromboemboli Atrial brillation

P ate et counts are usua y nor a . Patients usua y su er Other Immunoglobulins Multiple myeloma,

ro chronic ane ia an b ee ing e iso es. B ee ing cause occlusions Waldenström’s primary

by abnor a ities in ate et a hesiveness an rothro bin macroglobulinemia

ti e (P ) ay be seen, an the va ues o actor VIII ay Plasma proteins Cryoglobulinemia

be ow. Characteristica y, b oo s eci ens are escribe as Fibrin Cryo brinogenemia

having hy erviscosity that can contribute to the b ee ing

isor er. Red blood cells Polycythemia

Te rinci a cause o this ty e o ur ura is increase Platelets Thrombocytosis

intra u ina ressure. T is con ition can be observe Fat Fat emboli syndrome

aroun the ank es with ro onge stan ing an can be asso-

ciate with the resence o abnor a roteins in acrog ob- Reference: Greer JP, et al. Clinical Hematology, 14th ed, Philadelphia, PA:
Lippincott Williams & Wilkins, 2016.
u ine ias or hy erviscosity isor ers.