Page 466 - Clinical Hematology_ Theory _ Procedures ( PDFDrive )

Chronic eosinophilic leukemia Case studies

Describe characteristics of chronic eosinophilic leukemia. Analyze the patient history, clinical signs and symptoms, and labo-

ratory data for the stated case studies; answer the related critical
Mastocytosis thinking questions; and conclude the most likely diagnosis.

Explain the biology and pathogenesis of mastocytosis.

Myeloproliferative neoplasm, unclassi able NOTE:

(MPN-U) ■ indicates MLT and MLS core content

Describe the general characteristics of MPN-U. indicates MLT (optional) and MLS advanced content

GENERAL CHARACTERISTICS OF All o the MPNs involve ysregul tion t the ultipotent
MYELOPROLIFERATIVE NEOPLASMS he topoietic ste cell (CD34), with one or ore o the ol-
lowing sh re e tures:

Te myeloproliferative neoplasms (MPNs) (Box 23.1) re 1. Cytogenetic bnor lities

interrel te clon l he topoietic ste cell isor ers ch r- 2. Overpro uction o one or ore types o bloo cells with

cterize by excessive proli er tion o one or ore ture o in nce o tr ns or e clone

yeloi cell lines, or ex ple, gr nulocytes, erythrocytes, 3. Hypercellul r rrow or rrow f brosis

eg k ryocytes, or st cells. 4. T ro botic n /or he orrh gic blee ing

T e 2016 WHO revision o u ors n He topoietic 5. Extr e ull ry he topoiesis

n Ly phoi issues h s eight subtypes with new olec- 6. r ns or tion to cute leuke i

ul r genetic criteri . T e iscovery o ut tions in cruci l

genes istinguishes MPNs ro other neopl s s. Molecul r

n lysis is now incorpor te into the i gnosis workup o Relationship of the Myeloproliferative

MPNs. Rese rch olecul r v nces were c ppe by the Neoplasms

iscovery o the JAK2, J nus kin se 2 (JAK2V617F ut - MPNs re pri rily neopl s s o ults between 50 n

tion). T is iscovery llowe or ref ne ent o the cl ssif c - 70 ye rs o ge, but so e subtypes occur in chil ren. MPNs

tion o MPNs. h ve overl pping clinic l e tures.

In MPN, t le st one elev te cell line exhibits cytosis M jor ch nges in the new Worl He lth Org niz tion

with no cytopeni s in the st ble ph se. Initi lly, n MPN 2008 cl ssif c tion sche e inclu e:

is ch r cterize by hypercellular bone rrow with e ec-

tive he topoietic tur tion n incre se nu bers o 1. T e inclusion o JAK2 n MPL ut tions s clon l rk-

gr nulocytes, re bloo cells, n /or pl telets in the periph- ers or the i gnosis o PRV, essenti l thro bocytosis or

er l bloo . One type o MPN y evolve into nother type essenti l thrombocythemia (E ), n PMF

uring the course o the ise se. All the types o MPNs y 2. Mini iz tion o the role o re cell ss e sure ent or

evolve into cute leuke i . the i gnosis o PRV
3. Lowering o the pl telet count cuto level or i gnosis

o E ro 600 to 450 × 10 L
9

V ri tion in the p ttern o cellul r proli er tion n i -
BOX 23-1 erenti tion c n be expl ine by the clon l ut tion o plu-

ripotent ste cells with i erent line ge potenti ls. Fe tures

Major Categories of Myeloproliferative th t istinguish one c tegory ro nother re presente in

Neoplasms (MPNs) ble 23.1.

1. Chronic yelogenous leuke i , BCR-ABL1 positive Common Disorders of Hemostasis and

(CML) Coagulation

2. BCR-ABL1–neg tive MPN

. Polycythe i rubr ver (PRV) P tients with n MPN su er ro v rious il isor ers o

b. Essenti l thro bocytosis thro bocythe i (E ) he ost sis or co gul tion. An bnor l co gul tion ech -

c. Pri ry yelof brosis (PMF) nis is believe to be rel te to low-gr e, possibly secon -

. M stocytosis ry or o isse in te intr v scul r co gul tion (DIC),

e. Chronic neutrophilic leuke i (CNL) chronic st te o bnor l bloo co gul tion th t occurs even

. Chronic eosinophilic leuke i er tre t ent to re uce the pl telet count. Co gul tion

g. Myeloproli er tive neopl s -unknown (MPN-U) bnor lities inclu e prolonge ctiv te p rti l thro -

bopl stin ti e (AP ) n signif c ntly ecre se level o