Page 501 - Clinical Hematology_ Theory _ Procedures ( PDFDrive )

CHAPTER 24 ■ Myelodysplastic Syndromes and Myelodysplastic/Myeloproliferative Neoplasms 485

Clinical Signs and Symptoms MDS/ MPN Disorders

■ A history o in ections, blee ing, weight loss, or c r iov s- Chronic Myelom onocytic Leukem ia (CMML)

cul r sy pto s y be reporte . ■ It is clon l he tologic lign ncy th t is consi er bly

■ Ane i is co on initi l presenting sy pto . less requent th n the cute v riety.

■ Peripher l bloo s e rs usu lly e onstr te persistent
Laboratory Manifestations onocyte count gre ter th n 1 × 10 /L. T is is the h ll-
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■ MDS is ch r cteristic lly ni este by p ncytopeni in rk o CMML.
the peripher l bloo , yspl si o two or three cell lines ■ CMML evelops t e i n ge o 66 ye rs, n the

th t y initi lly be in just one cell line, n low leuke- le- e le r tio is 2.4:1.

ic bl st count in the bone rrow n peripher l bloo .

■ P ncytopeni occurs in ore th n 50% o p tients. Other Classi cations

Treatment ■ Atypic l chronic yeloi leuke i ( CML) exhibits

e tures o both yelo yspl stic n yeloproli er tive
■ re t ent consi er tions in MDS ust weigh the risk o isor ers t the ti e o i gnosis. It is ch r cterize by
ther py g inst the risk o proble s ssoci te with exist- leukocytosis with jority o neutrophils.

ing cytopeni s s well s the likelihoo n i inence o ■ Juvenile yelo onocytic leuke i (JMML) is isor er

leuke ic tr ns or tion. o chil hoo . It is ch r cterize by the proli er tion o

■ Chil ren tre te or MDS respon poorly to convention l gr nulocytic n onocytic line ges.
che other py.
■ Myelo yspl stic syn ro e/ yeloproli er tive neopl s ,

Relationship of Cytogenetics to Prognosis uncl ssif ble. T is neopl s eets the ef nition o
MDS/MPNs but oes not eet the criteri or CMML or

■ Surviv l is gener lly goo or p tients with re r ctory the other cl ssif c tion in this c tegory.

ne i s. ■ RARS- is new c tegory. Cytogenetic f n ings inclu e

■ Surviv l ti e or ll p tients with MDS is pproxi tely neg tivity or BCR-ABL n the presence o SF3B1 n

2 ye rs. JAK2 ut tions.

CASE STUDIES

Case Study 24.1 ( yspl stic) p ttern with nucle r:cytopl s ic synchrony.
M ny binucle r rubricytes were observe . More th n 15%
An el erly white wo n with history o ne i visite o the et rubricytes were si erobl sts.

her pri ry c re provi er bec use o incre sing shortness
o bre th, izziness, n severe tigue. She h no history ■ Critical Thinking Group Discussion Questions

o prior tre t ent with rugs or exposure to le or other 1. Wh t re si erobl sts?
toxins. Her physici n or ere CBC.
2. Wh t o si erobl sts represent?

■ Laboratory Data
RBC 3.52 × 10 /L 3. Wh t is the ost prob ble i gnosis in this c se?
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He tocrit 37%

He oglobin 12.4 g/ L Case Study 24.2

WBC 8.4 × 10 /L A 26-ye r-ol white n, with history o colitis 20 ye rs
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Pl telets 275 × 10 /L e rlier, visite his ily pr ctitioner bec use o tigue. He
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T e peripher l bloo s e r e onstr te 3+ polychro to- is t king e ic tion or hypertension (c ptopril) n c r-

phili , co rse b sophilic stippling, occ sion l P ppenhei er bonic nhy r se inhibitor or gl uco . He st tes th t he

bo ies, n occ sion l hyposeg ente neutrophils. en nu- rinks one to two beers y. He h col sever l weeks

cle te RBCs were seen per hun re leukocytes counte on go n h s not elt well since then. He h s no li estyle

the peripher l bloo i erenti l. Signif c nt thro bocytope- risk ctors. His physic l ex in tion reve le bruises in

ni with occ sion l gi nt pl telets w s lso note . unusu l loc tions (e.g., insi e his thigh) with no report o
ny injury. He h s sc ttere petechi e.

■ Follow-Up Laboratory Data

Reticulocyte count 4.3% ■ Laboratory Data
RBC 2.47 × 10 /L
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■ Bone Marrow Examination He tocrit 24%
T e bone rrow w s hypercellul r with erythroi hyper- He oglobin 7.7 g/ L

pl si . T e RBC precursors exhibite n bnor l growth WBC 4.4 × 10 /L
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(continued)