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ENJOY PATHOLOGY

Sreamboat, CO 

Hematopathology Notes

 Non-Neoplastic Hematology

Benign Red Blood Cell Disease

Abnormal RBC Shapes

  Normal RBC morphology

  Microcytic and hypochromic RBC

  Spherocytes

  Hereditary elliptocytosis

  Hereditary pyropoikilocytosis

  Ovalocyte

  Stomatocyte

  Teardrop cells

  Bite cells

  Schistocytosis

  Target cells

  Sickle cell

  Echinocytes (Burr cells)

  Acanthocytes (Spur cells)

 Hb CC

 Hb SC

  Thermal injury

  Rouleaux formation

  Cold agglutinins

 

Abnormal RBC Inclusions

 ▪ Basophilic stippling

 ▪ Howell-Jolly bodies

 ▪ Henz bodies

 ▪ Pappenheimer bodies

 ▪ Cabot rings

 ▪ Overlying platelet

 ▪ Malaria

 ▪ Babesiosis

 

Anemia

 ▪ Iron metabolism

 ▪ Laboratory tests for anemia

 ▪ Iron deficiency anemia

 ▪ Anemia of chronic disease

 ▪ Megaloblastic anemia

 ▪ Aplastic anemia

    ▫ Fanconi anemia

    ▫ Dyskeratosis congenita

    ▫ Shwachman-Diamond syndrome

 ▪ Paroxysmal nocturnal hemoglobinuria

 ▪ Sideroblastic anemia

 ▪ Pure red cell aplasia

     ▫ Diamond-Blackfan syndrome

     ▫ Congenital dyserythropoietic anemia

     ▫ Parvovirus

     ▫ Transient erythroblastopenia of childhood

 

Hemoglobinopathy

 ▪ Introduction

 ▪ Alpha chain disorders

     ▫ Alpha thalassemia

     ▫ Hb constant spring

     ▫ Hb G

 ▪ Beta chain disorders

     ▫ Beta thalassemia

     ▫ Hb S trait

     ▫ Hb S disease

     ▫ Hb C trait

     ▫ Hb C disease

     ▫ Hb C-Harlem

     ▫ Hb D

     ▫ Hb E

 ▪ Combined

     ▫ Hb S/C

     ▫ S/alpha thal carrier

     ▫ S/alpha thal trait

     ▫ S/β+ thal

     ▫ S/β0 thal

     ▫ HPFH (δ/β deletion)

     ▫ Hb Lepore (δ/β fusion)

     ▫ δβ thalassemia (δ/β deletion)

 

Benign White Blood Cell Disease

Abnormal Inclusions and Shapes

 ▪ Dohle body

 ▪ Toxic granulation (Hypergranularity)

 ▪ May Hegglin anomaly

 ▪ Chediak-Higashi syndrome

 ▪ Alder-Reilly anomaly

 ▪ Gaucher disease

 ▪ Niemann-Pick disease

 ▪ Hemophagocytosis

 ▪ Lupus Erythematosus cell

 ▪ Pelger-Huet anomaly

 ▪ Copper deficiency

 ▪ Histoplasma

 ▪ Leishmania

 ▪ Anaplasma phagocytophilum

 

Inherited Neutropenia

 ▪ Severe congenital neutropenia

 ▪ Kostmann’s disease

 ▪ Familial cyclic neutropenia

 ▪ Shwachman-Diamond syndrome

 ▪ Dyskeratosis Congenita

 ▪ Myelokathexis

 

Inherited Functional Abnormalities

 ▪ Chronic granulomatous disease

 ▪ Leukocyte adhesion molecule deficiency

 ▪ Myeloperoxidase deficiency

 

Reactive Lymphocytes

 ▪ Infectious mononucleosis

 ▪ Circulating immunoblasts in blood

 ▪ Lymphocytosis in pertussis

 ▪ Persistent polyclonal B-cell lymphocytosis

 ▪ CLL-like monoclonal B-lymphocytosis

 ▪ Hematogones

 

Platelet Disorders and von Willebrand Diseases

Structure of Platelet and von Willebrand Factor

 ▪ Platelet Function Assays

     ▫ Agonist-induced platelet aggregation

     ▫ Platelet function analyzer – 100 (PFA-100)

 

Quantitative Disorders (Thrombocytopenia)

 ▪ Acquired thrombocytopenia

     ▫ Immune thrombocytopenic purpura

     ▫ Neonatal alloimmune thrombocytopenia

     ▫ Post transfusion purpura

     ▫ Alloimmune thrombocytopenia

     ▫ Heparin-induced thrombocytopenia

     ▫ Thrombotic Thrombocytopenic Purpura

     ▫ Hemolytic Uremic Syndrome

     ▫ Pseudothrombocytopenia

 ▪ Congenital thrombocytopenia

     ▫ May Hegglin Anomaly

     ▫ Thrombocyopenia with absent radii

     ▫ Congenital amegakaryocytic

        thrombocytopenia

     ▫ X-linked thrombocytopenia

     ▫ Hermansky-Pudlak syndrome

 

 Qualitative Disorders

 ▪ Surface protein defect

    ▫ Bernard-Soulier syndrome

    ▫ Glanzmann thrombasthenia

 ▪ Storage pool deficiency: δ granules defect

    ▫ Wiscott Aldrich syndrome

    ▫ Chediak-Higashi disease

    ▫ Hermansky-Pudlak syndrome

 ▪ Gray platelet syndrome: α granules defect

    ▫ Cardiopulmonary bypass

    ▫ Quebec platelet disorder

 ▪ Acquired platelet dysfunction

 ▪ Aspirin: cyclooxygenase (COX) inhibition

 

von Willebrand Disease

 ▪ vWD, Type 1

 ▪ vWD, Type 2A

 ▪ vWD, Type 2B

 ▪ vWD, Type 2M

 ▪ vWD, Type 2N (Normandy)

 ▪ vWD, Type 3

 ▪ Pseudo-VWD (Platelet-type VWD)

 

 

  Lymphoid Disorders

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

 ▪ MAC 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)

 ▪ Primary mediastinal large B-cell lymphoma

 ▪ Intravascular large B-cell lymphoma

 ▪ ALK+ large B-cell lymphoma

 ▪ Primary CNS 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

 ▪ MGUS

 ▪ 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, NOS

 ▪ 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

 

Metastatic Tumors in Lymph Nodes

 ▪ Nodal Merkel cell carcinoma

 ▪ Metastatic lobular carcinoma of breast

 ▪ Metastatic nasopharyngeal carcinoma

 ▪ Metastatic seminoma

  Metastatic melanoma

 ▪ Metastatic rhabdomyosarcoma

 ▪ Myeloid sarcoma

  Bone Marrow Disorders
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

 

Neoplastic Marrow Disease

Myeloproliferative Neoplasms (MPN)

 ▪ Chronic myelogenous leukemia (CML)

 ▪ Chronic neutrophilic leukemia (CNL)

 ▪ Polycythemia vera (PV)

 ▪ Primary myelofibrosis (PMF)

 ▪ Essential thrombocythemia (ET)

 ▪ Chronic eosinophilic leukemia, NOS

 ▪ Mast cell disease (MCD)

 ▪ Myeloproliferative neoplasm, unclassifiable

 

Myelodysplastic Syndrome (MDS)

 ▪ Refractory cytopenia with unilineage dysplasia

 ▪ Refractory anemia with ring sideroblasts

 ▪ Refractory cytopenia with multilineage dysplasia

 ▪ Refractory anemia with excess blasts

 ▪ MDS associated with isolated del(5q)

 ▪ MDS, unclassifiable

 ▪ Refractory cytopenia of childhood

 ▪ MDS, therapy related

 

Myeloproliferative Neoplasms/Myelodysplastic Syndrome (MPN/MDS)

 ▪ Chronic myelomonocytic leukemia

 ▪ Atypical chronic myeloid leukemia (aCML)

 ▪ Juvenile myelomonocytic leukemia (JMML)

 ▪ Myelodysplastic/myeloproliferative neoplasm, 

    unclassifiable

 ▪ 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)

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

    ▫ Acute promyelocytic leukemia with t(15;17)

    ▫ AML with t(9;11)(p22;q23)

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

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

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

    ▫ 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/Dendritic Cell 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's 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

    ▫ Fibroblastic reticular cell tumor

    ▫ Indeterminate dendritic cell tumor

    ▫ Blastic Plasmacytoid Dendritic Cell Neoplasm

 ▪ Disseminated juvenile xanthogranuloma