Immature Hematopoietic Malignancies

37. Elastic NK-Cell Lymphoma 297

Werner Kempf, Reinhard Dummer, Dmitry V. Kazakov, and Günter Burg

Classification 297

Definition 297

Clinical Features 297

Histology 297

Immunohistochemistry 299

Molecular Biology 299

Treatment 300

Clinical Course and Prognosis 300

Comments 301

38. Precursor Lymphoblastic Leukemia/Lymphoma 303

38.1. Precursor T-Lymphoblastic Leukemia/Lymphoma 303

Werner Kempf, Dmitry V. Kazakov, and Günter Burg

Classification 303

Definition 303

Clinical Features 303

Histology 304

Immunohistochemistry 304

Molecular Biology 304

Treatment 304

Clinical Course and Prognosis 304

Differential Diagnosis 304

38.2. Precursor B-Lymphoblastic Leukemia/Lymphoma 307

Werner Kempf, Dmitry V. Kazakov, and Günter Burg

Classification 307

Definition 307

Clinical Features 307

Histology 308

Immunohistochemistry 309

Molecular Biology 309

Treatment 310

Clinical Course and Prognosis 310

Differential Diagnosis 310

39. Cutaneous Manifestations of Hodgkin Lymphoma 311

Marshall E. Kadin, Werner Kempf, and Gunter Burg

Introduction 311

Secondary Involvement of the Skin by Systemic Hodgkin Lymphoma 311

Skin Disorders Associated with Systemic Hodgkin Lymphoma 312

HIV-Associated Hodgkin Lymphoma 312

Primary Cutaneous Hodgkin Lymphoma 312

40. Cutaneous Pseudolymphomas 317

Günter Burg, Werner Kempf, and Dmitry V. Kazakov

Definition 317

Introduction 317

40.1. Cutaneous T-cell Pseudofymphoma 319

Günter Burg, Werner Kempf, and Dmitry V. Kazakov Lymphocytic Infiltration of Jessner-Kanof (Idiopathic Cutaneous

T-cell Pseudolymphoma) 319

Palpable Migratory Arciform Erythema 321

Lymphomatoid Contact Dermatitis 323

Actinic Reticuloid 323

Persistent Nodular Arthropod-Bite Reactions 323

40.2. Cutaneous B-cell Pseudolymphoma 325

Günter Burg, Werner Kempf, and Dmitry V. Kazakov

Lymphadenosis Benigna Cutis 325

Clinical Features 325

Histology 326

Immunohistochemistry 327

Molecular Biology 328

Differential Diagnosis 328

Comments 328

40.3. Acral Pseudolymphomatous Angiokeratoma of Children 331

Dmitry V. Kazakov, Günter Burg, and Werner Kempf

Definition 331

Clinical Features 331

Histological Features 331

Immunohistochemistry 332

Molecular Biology 333

Differential Diagnosis 333

Treatment 334

Clinical Course and Prognosis 334

Comments 334

40.4. Pseudolymphomatous Folliculitis 337

Dmitry V. Kazakov, Günter Burg, and Werner Kempf

Clinical Features 337

Histological Features 337

Immunohistochemistry 337

Molecular Biology 338

Differential Diagnosis 338

Treatment 338

Clinical Course 338

Comments 338

40.5. Cutaneous Inflammatory Pseudotumor 341

Dmitry V. Kazakov, Günter Burg, and Werner Kempf

Clinical Features 341

Histological Features 341

Immunohistochemistry 343

Molecular Biology 343

Differential Diagnosis 343

Treatment 344

Clinical Course 344

Comments 344

40.6. Castleman Disease 345

Dmitry V. Kazakov, Günter Burg, and Werner Kempf

Definition 345

Clinical Features 345

Histology 345

Immunohistochemistry 346

Molecular Biology 346

Treatment 346

Clinical Course and Prognosis 347

Differential Diagnosis 347

Comments 348

40.7. Pseudolymphomatous Infiltrates in the Context of Other Skin Disorders 349

Günter Burg and Werner Kempf

41. Parapsoriasis 351

Günter Burg, Werner Kempf, Reinhard Dummer, and Dmitry V. Kazakov

Historical Perspectives 351

Modern Perspectives 352

Commentary 352

41.1. Small Plaque Parapsoriasis 355

Günter Burg, Werner Kempf, Reinhard Dummer, and Dmitry V. Kazakov

Classification 355

Definition 355

Clinical Features 355

Histology 355

Immunohistochemistry 356

Molecular Biology 356

Treatment 357

Clinical Course and Prognosis 357

Differential Diagnosis 357

41.2. Large Plaque Parapsoriasis With and Without Poikiloderma 359

Gunter Burg, Werner Kempf, Reinhard Dummer, and Dmitry V. Kazakov

Large Patch Type Without Poikiloderma 359

Large Patch Type With Poikiloderma 360

42. Cutaneous T-cell Lymphoproliferative Disorders of Unknown Significance 363

Gunter Burg and Werner Kempf

42.1. Papuloerythroderma of Ofuji 363

Definition 363

Clinical Features 363

Histology 364

Immunohistochemistry 364

Treatment 365

Clinical Course and Prognosis 365

Differential Diagnosis 365

Comments 365

42.2. Clonal T-cell Disorders 367

Werner Kempf, Dmitry V. Kazakov, and Gunter Burg

Clonal Dermatitis 368

Lichen Planus and Psoriasis 368

Pityriasis Lichenoides 368

Persistent Pigmented Purpuric Dermatitis (PPPD) 370

Angiolymphoid Hyperplasia with Eosinophilia and Kimura Disease 371

43. Skin Involvement in Leukemias 373

Stanislaw Buechner

Introduction 373

43.1. Chronic Lymphocytic Leukemia, B-cell Type (B-CLL) 375

Stanislaw Buechner and Daniel W.P. Su

Clinical Features 375

Histology 376

Immunohistochemistry 377

Molecular Biology 377

43.2. Chronic Lymphocytic Leukemia, T-cell Type (T-CLL) 379

Stanislaw Buechner and Daniel W.P. Su

Clinical Features 379

Histology 380

Immunohistochemistry 381

43.3. Chronic Myeloid Leukemia (CML) 383

Stanislaw Buechner and Daniel W.P. Su

Clinical Features 383

Histology 384

Immunohistochemistry 384

43.4. Hairy-Cell Leukemia (HCL) 387

Stanislaw Buechner and Daniel W.P. Su

Clinical Features 387

Histology 388

Immunohistochemistry 389

43.5. Acute Lymphoblastic Leukemia (ALL) 391

Stanislaw Buechner and Daniel W.P. Su

Clinical Features 391

Histology 391

Immunohistochemistry 392

43.6. Acute (Myelo-) Monocytic Leukemia (AMoL, AMMoL) 393

Clinical Features 393

Histology 395

Immunohistochemistry 396

43.7. Acute Granulocytic (Myeloid) Leukemia (AML) 399

Stanislaw Buechner and Daniel W.P. Su

Clinical Features 400

Histology 400

Immunohistochemistry 401

43.8. Granulocytic Sarcoma (Chloroma) 403

Stanislaw Buechner and Daniel W.P. Su

Clinical Features 403

Histology 404

Immunohistochemistry 404

43.9. Large Granular Lymphocyte Leukemias 407

Dmitry V. Kazakov, Günter Burg, and Werner Kempf 43.10. Chronic Neutrophilic Leukemia 411

Dmitry V. Kazakov, Gunter Burg, and Werner Kempf

Classification 411

Definition 411

Clinical Features 411

Histology 412

Immunohistochemistry 412

Laboratory Findings 412

Cytogenetics 412

Treatment 412

Clinical Course and Prognosis 412

Differential Diagnosis 412

44. Paraneoplastic Cutaneous Reactions in Leukemia and Lymphomas 413

Werner Kempf, Reinhard Dummer, Dmitry V. Kazakov, and Günter Burg

Definition 413

Classic Paraneoplastic Diseases 413

Acute Febrile Neutrophilic Dermatosis 413

Vasculitis 415

Pathogenesis 416

Treatment 416

Comment 416

45. Histiocytic Disorders 419

Dmitry V. Kazakov, Günter Burg, and Werner Kempf

45.1. Langerhans Cell Histiocytosis (Histiocytosis X) 423

Dmitry V. Kazakov, Günter Burg, and Werner Kempf

Definition 423

Clinical Features 423

Histopathology 427

Immunohistochemistry 428

Electron Microscopy 428

Treatment 428

45.2. Indeterminate Cell Histiocytosis 431

Dmitry V. Kazakov, Günter Burg, and Werner Kempf

Definition 431

Clinical Features 431

Histology 431

Immunohistochemistry 432

Electron Microscopy 432

Treatment 433

Clinical Course and Prognosis 433

Comments 433

45.3. Sinus Histiocytosis with Massive Lymphadenopathy 435

Dmitry V. Kazakov, Günter Burg, and Werner Kempf

Definition 435

Clinical Features 435

Histology 436

Immunohistochemistry 437

Treatment 437

Clinical Course and Prognosis 437

Differential Diagnosis 438

Comments 438

45.4. Multicentric Reticulohistiocytosis (Lipoid Dermatoarthritis) 441

Dmitry V. Kazakov, Günter Burg, and Werner Kempf

Definition 441

Clinical Features 441

Histology 442

Immunohistochemistry 443

Treatment 443

Electron Microscopy 443

Clinical Course and Prognosis 444

Comments 444

45.5. Juvenile Xanthogranuloma 447

Dmitry V. Kazakov, Günter Burg, and Werner Kempf

Definition 447

Clinical Features 447

Histology 448

Immunohistochemistry 448

Treatment 449

Clinical Course and Prognosis 449

Comments 450

45.6. Benign Cephalic Histiocytosis 451

Dmitry V. Kazakov, Günter Burg, and Werner Kempf

Definition 451

Clinical Features 451

Histology 451

Immunohistochemistry 452

Electron Microscopy 452

Treatment 453

Clinical Course and Prognosis 453

Comments 453

45.7. Solitary Reticulohistiocytoma 455

Dmitry V. Kazakov, Günter Burg, and Werner Kempf

Definition 455

Clinical Features 455

Histology 455

Immunohistochemistry 456

Electron Microscopy 456

Clinical Course and Prognosis 457

Comments 457

45.8. Malignant Histiocytic Tumors 459

Dmitry V. Kazakov, Günter Burg, and Werner Kempf

Introduction 459

Malignant Histiocytic Tumors of the Skin 459

46. Etiology and Pathogenesis of Cutaneous Lymphomas 461

Günter Burg, Werner Kempf, Reinherd Dummer, Dmitry V. Kazakov, Frank O. Nestle, Antonio Cozzio, Sonja Michaelis, and Udo Dobbeling

46.1. Introduction 461

46.2. Genetics Factors 461

46.3. Environmental Factors 462

46.4. Infectious Factors 462

Human T-cell Lymphotropic Viruses 462

Epstein-Barr Virus and Other Herpesviruses 463

Borrelia burgdorferi 463

Superantigens 463

46.5. Immunological Factors 464

46.6. Pathogenetic Models of Cutaneous Lymphomagenesis 465

The Thymus Bypass Model 465

A Multifactorial Concept of the Pathogenesis of Cutaneous Lymphomas 466

47. Therapy of Cutaneous Lymphomas 475

47.1. Introduction 475

Gunter Burg, Werner Kempf, Reinhard Dummer, Antonio Cozzio, Sonja Michaelis, and Frank O. Nestle

Therapeutic Modalities 479

47.2. Topical Therapies 481

Günter Burg, Reinhard Dummer, Werner Kempf, Sonja Michaelis, Philippa Golling, and Frank O. Nestle

Nonaggressive Topical Therapies 481

Aggressive Topical Therapies 485

47.3. Systemic Therapies 497

Günter Burg, Reinhard Dummer, Werner Kempf, Sonja Michaelis, Philippa Golling, and Frank O. Nestle

Nonaggressive Systemic Therapies 497

Aggressive Systemic Therapies 504

47.4. Experimental Therapies 511

Günter Burg, Reinhard Dummer, Werner Kempf, and Frank O. Nestle

Topical Experimental Therapies 511

Systemic Experimental Therapies 514

Vaccination Therapy 519

47.5. Stage-Adjusted Treatment of Cutaneous Lymphomas: The Practical Approach . . . 525 Gunter Burg, Reinhard Dummer, Werner Kempf, Sonja Michaelis, Philippa Golling, and Frank O. Nestle

Cutaneous T-cell Lymphomas (CTCL) 525

Cutaneous B-cell Lymphomas 527

48. Prognosis 529

Gunter Burg, Werner Kempf, and Monika Hess Schmid

48.1. Prognostic Parameters in Cutaneous Lymphomas 529

Prognostic Parameters in Cutaneous T-cell Lymphomas 529

Prognostic Parameters in Cutaneous B-cell Lymphomas 534

The Prognostic Index 537

48.2. Survival Rates in Cutaneous Lymphomas 543

Gunter Burg and Werner Kempf

49. Concepts, Misconceptions, and Controversies in Cutaneous Lymphomas. . 547

Gunter Burg

Who Was First?: There Will Always Be Somebody "Before" 547

The Infinite Loop of Lymphoma Classification 547

Using the Same Words Does Not Mean Speaking the Same Language 548

The Emperor's New Clothes: Seeing Something That Is Not There 548

Pink and Blue vs. Red and Brown 548

What Makes the Lymphocyte Angry? 549

Which Target Do We Really Treat? 549

Index 551

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