About the author

Claudio Ortolani is an expert in the area of diagnosis of hematological malig­­nancies. Now retired, Dr Ortolani was Consultant in the Department of Clinical Pathology at Venice General Hospital, Venice, Italy. He is one of the founding members of the Italian Society for Cytometric Cell Analysis (ISCCA), of whose board he is currently a member. He has taught and lectured internationally on how to use flow cytometry to aid in diagnosing hematological diseases.

Foreword to the Second Edition xi
by Michael J. Borowitz

Foreword to the First Edition xii
by Maryalice Stetler-Stevenson

Foreword to the First Edition xiii
by Bruno Brando

Preface to the Second Edition xv

Preface to the First Edition xvi

Abbreviations xvii

1 Antigens 1

Clustered (CD) Antigens

CD1 3

CD2 5

CD3 8

CD4 17

CD5 21

CD7 24

CD8 26

CD10 30

CD11b 35

CD11c 38

CD13 40

CD14 44

CD15 46

CD16 49

CD19 52

CD20 55

CD22 59

CD23 61

CD24 64

CD25 66

CD26 67

CD27 69

CD28 70

CD30 71

CD33 73

CD34 77

CD38 79

CD43 81

CD45 82

CD45 Isoforms 87

CD49 90

CD56 93

CD57 96

CD61 97

CD62L 98

CD64 99

CD65 101

CD66c 102

CD71 103

CD79 104

CD81 107

CD103 108

CD117 110

CD123 112

CD138 113

CD200 114

CD305 116

CD307 (IRTA) Antigen Family 117

CD371 118

Non clustered (or primarily known with other names) antigens Bcl2 Protein 119

Chemokines and Chemokine Receptors 121

CRLF2 128

Cytotoxic Proteins 129

HLADR 130

Immunoglobulins 132

KIR, CD158 isoforms 136

Myeloperoxidase (MPO) 139

NG2 140

PCA1 141

ROR1 141

SLAM Molecules and SLAMassociated Protein (SAP) 142

SOX11 144

Tcell Receptor (TCR) 145

Terminal Deoxynucleotidyltransferase (TdT) 148

Tolllike Receptors (TLR) 150

VS38 151

ZAP70 152

2 Diseases 155

Myeloproliferative neoplasms 157

Chronic myeloid leukemia (CML) 157

Myeloproliferative neoplasms other than CML 160

Chronic neutrophilic leukemia (CNL) 160

Polycythemia vera (PV) 160

Primary myelofibrosis (PMF) 160

Essential thrombocythemia (ET) 160

Chronic eosinophilic leukemia (CEL) 161

Mastocytosis 162

Acute mastcell leukemia (AMCL) 162

Chronic mastcell leukemia (CMCL) 163

Myelomastocytic leukemia (MML) 163

Myelodysplastic/myeloproliferative neoplasms 164

Chronic myelomonocytic leukemia (CMML) 164

Other myelodysplastic/myeloproliferative neoplasms and related conditions 167

Juvenile myelomonocytic leukemia (JMML) 167

Atypical CML bcr/abl negative (ACML) 167

RASassociated autoimmune leukoproliferative disorder (RALD) 167

Myelodysplastic syndromes 168

Myeloid neoplasms with germline predisposition 171

Acute myeloid leukemias 172

AMLs with recurrent genetic anomalies 173

AMLs with chromosomal anomalies 173

AMLs with gene mutations 180

Relationships between genotype and phenotype in cases of AML not recognized as separate entities in WHO 2017 181

AMLs with myelodysplasiärelated changes (AMLMRC) 182

AMLs not otherwise specified 182

AML with minimal differentiation 182

AML without maturation 183

AML with maturation 183

Acute myelomonocytic leukemia (AMMoL) 183

Acute monoblastic and monocytic leukemia (AMoL) 184

Pure erythroid leukemia (PEL) 185

Acute megakaryoblastic leukemia (AMKL) 186

Acute basophilic leukemia (ABL) 188

Myeloid proliferations associated with Down syndrome 188

Transient abnormal myelopoiesis (TAM) 189

AMLs in patients with Down syndrome 189

Blastic plasmacytoid dendritic cell neoplasm (BPDCN/PDCL) 189

Acute leukemias with ambiguous lineage attribution (ALAL) 192

Acute undifferentiated leukemias (AUL) 192

Mixed phenotype acute leukemias (MPAL) 192

Neoplastic diseases of B and T lymphatic precursors 194

B lymphoblastic leukemia/lymphoma, not otherwise specified (BALL/LBLnos) 195

B lymphoblastic leukemia/lymphoma with recurrent genetic anomalies 197

Relationships between genotype and phenotype in cases of BALL not recognized as separate entities in WHO 2017 201

T lymphoblastic leukemia/lymphoma (TALL/LBL) 202

Early Tcell precursor lymphoblastic leukemia (ETPALL) 205

NK lymphoblastic leukemia/lymphoma (NKALL/LBL) 205

Neoplastic diseases of mature B cells 206

Chronic lymphocytic leukemia/small

lymphocytic lymphoma (BCLL/SLL) 206

Familial BCLL 215

Richter syndrome 215

Monoclonal Bcell lymphocytosis (MBL) 216

CLLlike monoclonal B lymphocytosis 216

NonCLLlike monoclonal B lymphocytosis 216

Bcell prolymphocytic leukemia (BPLL) 216

Lymphoplasmacytic lymphoma (LPL) 218

Heavy chain disease (HCD) 221

heavy chain disease 222

heavy chain disease 222

heavy chain disease 222

Hairy cell leukemia (HCL) 222

Hairy cell leukemia, variant (HCLv) 226

Hairy cell leukemia, Japanese variant (HCLJ) 227

Splenic diffuse red pulp lymphoma (SDRPL) 227

Marginal zone lymphomas (MZL) 228

Nodal marginal zone lymphoma (NMZL) 229

Splenic marginal zone lymphoma (SMZL) 230

Extranodal marginal zone lymphoma (EMZL/MALToma) 232

Clonal Bcell lymphocytosis with MZLlike phenotype (CBLMZ) 233

Follicular lymphoma (FCL) 234

Testicular follicular lymphoma 237

Duodenal type follicular lymphoma 237

Pediatric type follicular lymphoma 237

Primitive cutaneous follicular lymphoma (PCFL) 237

Large Bcell lymphoma with IRF4 rearrangement 237

Mantlecell lymphoma (MCL) 237

Blastic mantlecell lymphoma (BMCL) 240

Leukemic non nodal mantlecell lymphoma 240

DLBCL not otherwise specified (DLBCLnos) 240

CD5(+) diffuse large cell lymphoma (CD5(+) DLBCL) 243

Tcell/histiocyterich Bcell lymphoma (THRLBCL) 243

Primary DLBCL of the CNS (PCNSL) 244

Primary cutaneous DLBCL, "leg type" 244

EBV(+) DLBCLnos 244

DLBCL associated with chronic inflammation (PAL) 245

Fibrin associated DLBCL 245

Lymphomatoid granulomatosis (LYG) 245

Primary mediastinal Bcell lymphoma (PMBCL) 245

Intravascular large Bcell lymphoma (IVBCL) 246

ALKpositive large cell lymphoma (ALK(+) LBCL) 246

Plasmablastic lymphoma (PBL) 247

Primary effusion lymphoma (PEL) 247

HHV8associated lymphoproliferative disorders 247

HHV8positive DLBCL 248

HHV8positive germinotropic lymphoproliferative disorder 248

Burkitt lymphoma (BL) 248

Burkitt leukemia with immature phenotype 250

Burkittlike lymphoma with 11q aberrations 251

Highgrade Bcell lymphoma (HGBL) 251

Plasma cell neoplasms 251

Monoclonal gammopathies of undetermined significance (MGUS) 253

Multiple myeloma (MM) 253

Plasma cell leukemia (PCL) 257

Neoplastic diseases of mature T and NK cells 258

Tcell prolymphocytic leukemia (TPLL) 258

Tcell large granular lymphocytic leukemia (TLGL) 261

Chronic lymphoproliferative disorders of NK cells (CLPDNK/CNKL) 263

Aggressive NKcell leukemia (ANKL) 266

Adult Tcell leukemia/lymphoma (ATLL) 266

Extranodal NK/T-cell lymphoma, "nasal type" (ENKTL) 269

Intestinal Tcell lymphomas (ITCL) 270

Enteropathyassociated Tcell lymphoma (EATCL) 270

Monomorphic epitheliotropic intestinal Tcell lymphoma (MEITL) 272

Indolent gastrointestinal T lymphoproliferative disorder (indolent GI TLPD) 273

Hepatosplenic Tcell lymphoma (HSTCL) 273

Subcutaneous panniculitislike Tcell lymphoma (SPTCL) 275

Mycosis fungoides (MF) 275

Sezary syndrome (SS) 277

Primary cutaneous CD30(+) lymphoproliferative disorders 279

Lymphomatoid papulosis (LyP) 279

Primary cutaneous anaplastic Tcell lymphoma (pcALCL) 279

Primary cutaneous peripheral Tcell lymphoma (PTCL) 280

Primary cutaneous TCR(+) Tcell lymphoma (PCGDTCL) 280

Primary cutaneous CD8(+) aggressive epidermotropic cytotoxic Tcell lymphoma (PCAETL) 280

Primary cutaneous acral CD8(+) Tcell lymphoma (PCATCL) 280

Primary cutaneous lymphoma of the medium/small CD4(+) T cells (PCSMTCL) 281

Peripheral Tcell lymphoma, not otherwise specified (PTCLnos) 281

Nodal lymphomas of follicular Thelper derivation 283

Angioimmunoblastic Tcell lymphoma (AITL) 283

Follicular Tcell lymphoma (FTCL) 285

Nodal PTCL with follicular Thelper phenotype 285

Anaplastic large cell lymphoma ALK(+) (ALCL ALK(+)) 285

Anaplastic large cell lymphoma ALK() (ALCL ALK()) 288

Breast implant-associated anaplastic large cell lymphoma (biaALCL) 288

Hodgkin lymphomas 289

Classic Hodgkin lymphoma (CHL) 289

Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) 290

Neoplastic diseases of histiocytic and dendritic cells 291

Histiocytic sarcoma (HS) 292

Langerhans cell histiocytosis (LCH) 292

Indeterminate dendritic cell tumor (IDCT) 292

Interdigitating dendritic cell sarcoma (IDCS) 292

Follicular dendritic cell sarcoma (FDCS) 292

Erdheim-Chester disease (EDC) 292

3 Appendix 293

Acute leukemias not recognized by the 2017 WHO classification 294

Acute leukemia of myeloid/NK precursors (M/NKAL) 294

Acute leukemia of myeloid dendritic cells (MDCL) 294

Acute leukemia of Langerhans cells 294

Composite lymphomas 294

Hypereosinophilic syndrome (HES), lymphocyte variant 295

Indolent T lymphoblastic proliferations (iTLBP) 295

Polyclonal lymphocytoses of B lymphocytes 298

Persistent polyclonal Bcell lymphocytosis (PPBL) 298

Persistent polyclonal CD5(+) Bcell lymphocytosis 298

Persistent polyclonal Bcell lymphocytosis, Japanese (hairy)...