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HEMATOPATHOLOGY NOTES

Indian Peaks, CO, 09/03/2016

Lymph Node/Lymphoid Pathology

Bone Marrow Pathology

Reactive Lymphadenopathy

Normal Lymph Node

 ▪ B-cell development

 ▪ Anatomy and cells

 ▪ Immunostaining patterns

 

Primarily Follicular Pattern

 ▪ Reactive follicular hyperplasia

 ▪ Progressive transformation of germinal centers

 ▪ Human immunodeficiency virus lymphadenitis

 ▪ Toxoplasmosis lymphadenitis

 ▪ Kimura disease

 ▪ Castleman disease

    ▫ Hyaline vascular variant

    ▫ Plasma cell variant, localized

    ▫ Plasma cell variant, systemic

 ▪ Rheumatoid arthritis

 ▪ IgG4-related lymphadenopathy

 ▪ Syphilitic lymphadenitis (Luetic lymphadenitis)

 

Primarily Paracortical Pattern

 ▪ Acute infectious mononucleosis

 ▪ Cytomegalovirus infection

 ▪ Herpes simplex lymphadenitis

 ▪ Dermatopathic lymphadenitis

 ▪ Postvaccinial lymphadenitis

 ▪ Drug induce lymphadenopathy (Dilantin)

 ▪ Nonspecific reactive paracortical hyperplasia

 

With Extensive Necrosis

 ▪ Systemic lupus erythematosus

 ▪ Kikuchi-Fujimoto lymphadenitis

 ▪ Kawasaki disease

 ▪ Complete necrosis/infarction

 

Primarily Sinus Pattern

 ▪ Reactive sinus histiocytosis

 ▪ Rosai-Dorfman disease

 ▪ Hemophagocytic syndrome

 ▪ Whipple's disease

 ▪ Exogenous or endogenous lipids

 

Granulomatous Reaction

 ▪ Sarcoidosis

 ▪ Cat-scratch disease

 ▪ Mesenteric yersinial lymphadenitis

 ▪ Lymphogranuloma venereum

 ▪ Mycobacterium avium-intrcellulare lymphadenitis

 ▪ Fungal infection

 

Alteration of  Lymph Node Framework

 ▪ Bacillary angiomatosis

 ▪ Kaposi sarcoma

 ▪ Inflammatory pseudotumor

 ▪ Vascular transformation

 ▪ Palisaded myofibroblastoma

 

Mature B-Cell Neoplasms

Small B-Cell Lymphomas/Leukemias

 ▪ Chronic lymphocytic leukemia/small lymphocytic lymphoma

 ▪ B-cell prolymphocytic leukemia

 ▪ Lymphoplasmacytic lymphoma /Waldenström's macroglobulinemia

 ▪ Hairy cell leukemia

 ▪ Hairy cell leukemia variant

 ▪ Follicular Lymphoma

    ▫ Conventional follicular lymphoma

    ▫ Pediatric follicular lymphoma

    ▫ Primary intestinal follicular lymphoma

    ▫ Intrafollicular neoplasia (in situ FL)

 ▪ Marginal zone lymphoma (MZL)

    ▫ Splenic marginal zone lymphoma

    ▫ Nodal marginal zone lymphoma

    ▫ MALT lymphoma

    ▫ Pediatric nodal marginal zone lymphoma

 ▪ Mantle cell lymphoma

 

Primary Cutaneous B-Cell Lymphoma

 ▪ Primary cutaneous marginal zone lymphoma

 ▪ Primary cutaneous follicle center lymphoma

 ▪ Cutaneous large B-cell lymphoma–leg type

 

Diffuse Large B-Cell Lymphomas (DLBCL) and Subtypes

 ▪ Primary mediastinal (thymic) large B-cell lymphoma

 ▪ Intravascular large B-cell lymphoma

 ▪ ALK+ large B-cell lymphoma

 ▪ Primary central nervous system large B-cell lymphoma

 ▪ Primary testicular large B-cell lymphoma

 ▪ de novo CD5+ DLBCL

 ▪ EBV+ DLBCL of the elderly

 ▪ Pyothorax-associated lymphoma

 ▪ Plasmablastic lymphoma

 ▪ HHV8 and EBV associated germinotropic lymphoproliferative disorder

 ▪ Large B-cell lymphoma arising in HHV8-associated multicentric Castleman's disease

 

Lymphomatoid granulomatosis (LYG)

Burkitt Lymphoma

Double Hit Lymphomas

 

Plasma Cell Neoplasm

 ▪ Monoclonal gammopathy of undetermined significance

 ▪ Plasma cell myeloma

 ▪ Solitary plasmacytoma of bone

 ▪ Extraosseous or extramedullary plasmacytoma

 ▪ Osteosclerotic myeloma

 

Hodgkin Lymphoma

 ▪ Nodular lymphocyte-predominant HL

 ▪ Classic Hodgkin lymphoma

    ▫ Nodular sclerosis subtype

    ▫ Mixed cellularity subtype

    ▫ Lymphocyte-rich subtype

    ▫ Lymphocyte-depleted subtype


Mature T-Cell or NK Cell Neoplasms

 ▪ T-cell prolymphocytic leukemia

 ▪ Adult T-cell leukemia/lymphoma

 ▪ Hepatosplenic T-cell lymphoma

 ▪ Peripheral T-cell lymphoma, not otherwise specified

 ▪ Angioimmunoblastic T-cell lymphoma

 ▪ Anaplastic large cell lymphoma, ALK positive

 ▪ Anaplastic large cell lymphoma, ALK negative

 ▪ Enteropathy-associated T-cell lymphoma

    ▫ Classic type

    ▫ Type II

 ▪ Extranodal NK/T-cell lymphoma, nasal type

 ▪ T-cell large granular lymphocytic leukemia

 ▪ Chronic lymphoproliferative disorder of NK Cells

 ▪ Aggressive NK-cell leukemia/lymphoma

 

Primary Cutaneous T-Cell Neoplasms

 ▪ Mycosis Fungoides/Sezary syndrome

 ▪ Lymphomatoid Papulosis

 ▪ Cutaneous anaplastic large cell lymphoma

 ▪ Subcutaneous panniculitis-like T-cell lymphoma

 ▪ Primary cutaneous gamma-delta T-cell lymphoma

 ▪ Primary cutaneous CD8-positive aggressive epidermotropic cytotoxic T-cell lymphoma

 ▪ Primary cutaneous CD4+ small/medium T-cell lymphoma

 

EBV Associated NK or T-Cell Lymphoproliferative Disorders in Childhood

 ▪ Chronic active EBV infection of T cells or NK Cells

 ▪ Mosquito bite hypersensitivity

 ▪ Hydroa bacciniforme

 ▪ Hydroa vacciniforme–like T-cell lymphoma

 ▪ Systemic T-cell lymphoproliferative disease of childhood

Reactive Bone Marrow Diseases

 ▪ Normal hematopoiesis and cell morphology

 ▪ Megaloblastic anemia

 ▪ Iron deficiency anemia

 ▪ Anemia of chronic disease

 ▪ Serous degeneration

 ▪ Changes in osteoblastic activities

 ▪ Fungal infection

 ▪ Mycobacterial infection

 ▪ Rickettsial infection

 ▪ Parvovirus infection


Primary Neoplastic Marrow Neoplastic Diseases

Myeloproliferative Neoplasms (MPN)

 ▪ Chronic myelogenous leukemia (CML)

 ▪ Chronic neutrophilic leukemia (CNL)

 ▪ Polycythemia vera (PV)

 ▪ Primary myelofibrosis (PMF)

 ▪ Essential thrombocythemia (ET)

 ▪ Chronic eosinophilic leukemia, not otherwise specified (CEL, NOS)

 ▪ Mast cell disease (MCD)

 ▪ Myeloproliferative neoplasm, unclassifiable (MPN, U)

 

Myelodysplastic Syndrome (MDS)

 ▪ Refractory cytopenia with unilineage dysplasia (RCUD)

 ▪ Refractory anemia with ring sideroblasts (RARS)

 ▪ Refractory cytopenia with multilineage dysplasia (RCMD)

 ▪ Refractory anemia with excess blasts (RAEB-1, RAEB-2)

 ▪ MDS associated with isolated del(5q)

 ▪ MDS, unclassifiable

 ▪ Refractory cytopenia of childhood (RCC; provisional)

 ▪ MDS, therapy related

 

Myeloproliferative Neoplasms/Myelodysplastic Syndrome (MPN/MDS)

 ▪ Chronic myelomonocytic leukemia (CMML)

 ▪ Atypical chronic myeloid leukemia, BCR-ABL1 negative (aCML)

 ▪ Juvenile myelomonocytic leukemia (JMML)

 ▪ Myelodysplastic/myeloproliferative neoplasm, unclassifiable (MDS/MPN, U)

 ▪ Provisional entity: refractory anemia with ring sideroblasts associated with

    marked thrombocytosis (RARS-T)

 

Eosinophilia and Chronic Eosinophilic Leukemia, Including Myeloid/Lymphoid Neoplasms with Eosinophilia and Abnormalities of PDGFRA, PDGFRB, and FGFR1

 ▪ Chronic Eosinophilic Leukemia and Other Myeloid and Lymphoid Neoplasms

     Associated with PDGFRA Rearrangement

 ▪ Myeloid Neoplasms Associated with PDGFRB Rearrangement

 ▪ Lymphoid and Myeloid Neoplasms Associated with FGFR1 Rearrangement

 

Acute Myeloid Leukemia (AML)

 ▪ AML with Recurrent Genetic Abnormalities

    ▫ AML with t(8;21)(q22;q22) (RUNX1-RUNX1T1)

    ▫ AML with inv(16)(p13.1q22) or t(16;16)(p13.1;q22) (CBFB-MYH11)

    ▫ Acute promyelocytic leukemia with t(15;17)(q22;q12) (PML-RARA)

    ▫ AML with t(9;11)(p22;q23) (MLLT3-MLL)

    ▫ AML with t(6;9)(p23;q34) (DEK-NUP214)

    ▫ AML with inv(3)(q21q26.2) or t(3;3)(q21;q26.2) (RPN1-EVI1)

    ▫ AML (megakaryoblastic) with t(1;22)(p13;q13) (RBM15-MKL1)

    ▫ Provisional entity: AML with mutated NPM1

    ▫ Provisional entity: AML with mutated CEBPA

 ▪ AML with Myelodysplasia-Related Changes

 ▪ Therapy-Related Myeloid Neoplasms

 ▪ AML, not otherwise specified

    ▫ AML with minimal differentiation (M0)

    ▫ AML without maturation (M1)

    ▫ AML with maturation (M2)

    ▫ Acute myelomonocytic leukemia (M4)

    ▫ Acute monoblastic/monocytic leukemia (M5)

    ▫ Acute erythroid leukemia (M6)

        · Pure erythroid leukemia

        · Erythroleukemia, erythroid/myeloid

    ▫ Acute megakaryoblastic leukemia (M7)

    ▫ Acute basophilic leukemia

    ▫ Acute panmyelosis with myelofibrosis

 ▪ Myeloid Proliferations Related to Down Syndrome

    ▫ Transient abnormal myelopoiesis

    ▫ AML associated with Down syndrome


B-Cell Acute Lymphoblastic Leukemia /Lymphoblastic Lymphoma
T-Cell Acute Lymphoblastic Leukemia /Lymphoblastic Lymphoma


Histiocytic Proliferations

Nonneoplastic Histiocytic Proliferations

 ▪ Reactive sinus histiocytosis

 ▪ Sinus histiocytosis with massive lymphadenopathy

 ▪ Hemophagocytic syndromes

 ▪ Familial hemophagocytic lymphohistiocytosis

 ▪ Secondary hemophagocytic syndromes

 ▪ Storage disorders

    ▫ Neimann-Pick disease

    ▫ Gaucher disease

    ▫ Tangier disease

 

Histiocytic and Dendritic Cell Neoplasms

 ▪ Macrophage/histiocytic neoplasm

    ▫ Histiocytic sarcoma

 ▪ Dendritic cell neoplasms

    ▫ Langerhans cell histiocytosis

    ▫ Langerhans cell sarcoma

    ▫ Interdigitating dendritic cell sarcoma

    ▫ Follicular dendritic cell sarcoma

    Blastic Plasmacytoid Dendritic Cell Neoplasm

    ▫ Fibroblastic reticular cell tumor

    ▫ Indeterminate dendritic cell tumor

 ▪ Disseminated juvenile xanthogranuloma