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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_2768_Библиотеки_им_академика_М_И_Перельмана
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ChAPTER 17 Myeloproliferative Neoplasms
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CHRONIC MYELOGENOUS LEUKEMIA, BCR-ABL1 POSITIVE
167
A
FIGURE 17.1A Peripheral blood. Note immature baso-
phils and eosinophil (original size ×500).
C
B
FIGURE 17.1B Peripheral blood. Arrow shows a
micromegakaryocyte.
D
FIGURE 17.1C A spectrum of granulocytes, including
multiple myelocytes, bands, and an immature
basophil ([Bone marrow] BM×500).
NOTE: BCR-ABL positivity must be present for diagnosis.
FIGURE 17.1D Multiple eosinophils, some of them
immature (BM×500).
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SECTION 4 Leukocytes
MORPHOLOGY
Peripheral Blood: Chronic phase*
LEUKOCYTES
Marked leukocytosis (12-1000 × 10
• Spectrum of myeloid cells with a predominance of myelocytes and segmented neutrophils
• Myeloblasts less than 5%
•± Pseudo-Pelger-Huët cells
• Basophilia
• Eosinophilia
•± Monocytosis
• Leukocyte alkaline phosphatase (LAP) markedly decreased (Figure 17.2)
ERYTHROCYTES
Normal or decreased in number
PLATELETS
• Normal or increased
•± Circulating micromegakaryocytes
Bone Marrow: Chronic phase*
• Hypercellular with expansion of granulocyte pool
• Myeloid:Erythroid (M:E) ratio increased
• Myeloblasts less than 5%
• Megakaryocytes normal to increased; may be immature and/or atypical
• ± Pseudo-Gaucher cells (see Figure 22.1A)
• ± Sea blue histiocytes (see Figure 22.5A)
9
/L)
*Before development of tyrosine kinase inhibitors for treatment, CML would progess through phases, from chronic to accelerated to blast phase, with increasing numbers of blasts, basophils, micromegakaryocytes, and dysplasia. (See a hematology
textbook, such as Rodak’s Hematology, for a complete discussion of the progression of CML.)
ChAPTER 17 Myeloproliferative Neoplasms
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169
LEUKOCYTE ALKALINE PHOSPHATASE (LAP)
A
FIGURE 17.2A LAP (0) (PB ×1000).
C
FIGURE 17.2C LAP (2 +) (PB ×1000).
D
FIGURE 17.2B LAP (1 +) (PB ×1000).
3
FIGURE 17.2D LAP (3 +, 4 +) (PB ×1000).
4
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SECTION 4 Leukocytes
LAP is an enzyme found in secondary granules of neutrophils. LAP activity is scored from 0 to 4+ in the mature
segmented neutrophils and bands. One hundred cells are scored and results are added together for the LAP
score. A normal score is approximately 20 to 100. Low (< 20) scores may be found in untreated CML, paroxysmal
nocturnal hemoglobinuria, sideroblastic anemia, and myelodysplastic syndromes. Higher scores may be found
in leukemoid reactions (Table 17.2).
GENETICS
BCR-ABL positivity must be present for diagnosis.
TABLE 17.2
Comparison of Chronic Myelogenous Leukemia (CML) and Leukemoid Reaction in
Peripheral Blood
CML Leukemoid Reaction
Neutrophils Increased with immature cells;
peaks at myelocyte and
segmented neutrophil stages
Eosinophils Increased with immature forms Normal
Basophils
Platelets Abnormal number Normal
Abnormal morphology
Dyspoiesis Present Absent, but may be reactive
Leukocyte alkaline
phosphatase
BCR/ABL1 Positive Negative
Markedly decreased Increased
Increased with immature forms;
orderly progression of maturation
stages with no peaks
changes
⁎
*
Leukemoid reaction: persistent neutrophilic leukocytosis above 50,000/uL when the cause is other than
leukemia.
ChAPTER 17 Myeloproliferative Neoplasms
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POLYCYTHEMIA VERA
171
A
FIGURE 17.3A Peripheral blood (original
magnication ×1000).
C
FIGURE 17.3C Bone marrow (original
magnication ×1000).
B
FIGURE 17.3B Bone marrow (original
magnication ×500).
MORPHOLOGY
Peripheral Blood
LEUKOCYTES
Normal or increased
• Neutrophilia with few metamyelocytes, rare
myelocytes
• Promyelocytes and myeloblasts extremely rare
•± Eosinophilia and/or basophilia
ERYTHROCYTES
Absolute erythrocytosis
• Hemoglobin greater than 18.5g/dL in male
individuals
• Hemoglobin greater than 16.5g/dL in female
individuals
PLATELETS
Normal or increased
Bone Marrow
• Hypercellular with panmyelosis
• M:E ratio usually normal
• Megakaryocytes may be abnormal in size and
morphology
GENETICS
JAK2 V617F or other JAK2 mutation is found in more
than 95% of cases, but is not specic for PV.
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SECTION 4 Leukocytes
ESSENTIAL THROMBOCYTHEMIA
A
FIGURE 17.4A Peripheral blood (original
magnication ×1000).
MORPHOLOGY
Peripheral Blood:
LEUKOCYTES
• Normal or slightly increased
• Normal maturation and distribution
ERYTHROCYTES
Normal or slightly decreased
PLATELETS
• Marked sustained thrombocytosis
• Variation in size from tiny to giant
Bone Marrow: Hypercellular with expan-
sion of the megakaryocyte pool
• Large megakaryocytes with abundant
cytoplasm
• May exhibit hyperlobulation
• Mild granulocytic hyperplasia
• Mild erythrocytic hyperplasia
GENETICS
No specic genetic or cytogenetic abnormality, but up to 50% of cases carry JAK2 V617F.
JAK2 is also found in polycythemia vera and
primary myelobrosis.
B
FIGURE 17.4B Bone marrow (original
magnication ×500).
C
FIGURE 17.4C Bone marrow showing abnormal
hyperlobulated megakaryocyte (original
magnication ×1000).
ChAPTER 17 Myeloproliferative Neoplasms
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PRIMARY MYELOFIBROSIS
173
A
FIGURE 17.5A Peripheral blood (×1000; subtle
changes).
B
FIGURE 17.5B Peripheral blood (×1000; more
advanced case).
MORPHOLOGY
Peripheral Blood
LEUKOCYTES
Normal, increased, or decreased
• Immature granulocytes
• Less than 5% blasts
ERYTHROCYTES
Normal or decreased
• Teardrop cells common, nucleated erythrocytes, polychromasia
PLATELETS
Low, normal, or increased
• May be giant with atypical shapes
• Abnormal granulation
• ± Circulating micromegakaryocytes
Bone Marrow: Aspiration attempts often result in a dry tap; biopsy results exhibit marked brosis with islands
of hematopoietic activity and pockets of clumped megakaryocytes
GENETICS
No specic genetic or cytogenetic abnormality, but up to 50% of cases carry JAK2 V617F. JAK2 is also found in
polycythemia vera and essential thrombocythemia.
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CHAPTER 18
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MYELODYSPLASTIC
SYNDROMES
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SECTION 4 Leukocytes
yelodysplastic syndromes (MDSs) are acquired clonal hematological
disorders characterized by normocellular/hypercellular marrow, ineffective
M
blood, reecting maturation defects in erythrocytes, leukocytes, and/or platelets.
morphologic ndings are common to all types of MDS: the presence of progressive
cytopenias in spite of a cellular bone marrow and dyspoiesis in one or more cell lines.
Subtypes of the 2017 World Health Organization (WHO) classication of MDSs
are listed in Box 18.1
hematopoiesis that leads to progressive cytopenia, and dysplasia in peripheral
MDSs are heterogeneous and have a multitude of expressions; however, two
BOX 18.1
World Health Organization Classication of
Myelodysplastic Syndromes (2017)
Refractory cytopenia with unilineage dysplasia
Refractory anemia with ring sideroblasts
Refractory cytopenia with multilineage dysplasia
Myelodysplastic syndrome with excess blasts
Myelodysplastic syndrome with isolated del(5q)
Myelodysplastic syndrome, unclassiable
Childhood myelodysplastic syndrome
Refractory cytopenia of childhood
From Swerdlow SH, Campo E, Harris NL, Pileri SA, Stein H, Thiele J (Eds): WHO
classication of tumours of haematopoietic and lymphoid tissues, (Revised
4th edition), IARC: Lyon 2017.
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