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SBL Block 1 > White Blood Cells > Flashcards

White Blood Cells Flashcards

1
Q

Neutropenia/Agranulocytosis

Pathogenesis (6) Clinical Feature

A

Agranulocytosis is clinically significant neutropenia*

Inadequate Granulopoiesis:
Suppression of stem cells
Drug suppression of precursors (most common)
Ineffective hematopoiesis

Accelerated Destruction:
Immune mediated injury
Splenomegaly (sequestration)
Increased peripheral utilization

Infections causing mucosal ulcerations

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2
Q

Leukocytosis

Etiologies (4) Types with Causes (5)

A

Increased production in marrow (chronic disorders)
Increased release from marrow stores (infection)
Decreased margination (exercise)
Decreased extravasation into tissues (glucocorticoids)

Neutrophilic: Acute bacterial infection, inflammation
Eosinophilic: Allergies, Autoimmune Disorders
Basophilic: myeloproliferative disorders
Monocytosis: Chronic infections
Lymphocytosis: Chronic infections, Viral infections

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3
Q 
Lymphadenitis
Acute Etiologies (4)
Chronic Morphology (4)
A

Cervical: Infections of teeth or tonsils
Axillary/Inguinal: Extremity infections
Mesenteric: Acute appendicitis
Generalized: Systemic viral infections and bacteremia

Follicular Hyperplasia (humoral germinal centers)
Tingible Body Macrophages seen between GC's
Paracortical Hyperplasia (T cell response)
Sinus Histiocytosis (sinuses draining cancer)
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4
Q

Hemophagocytic Lymphohistiocytosis

Pathogenesis (4) Labs (4) Clinical Features (3) Treatment (2)

A

Activation of macrophages and CD8+ CTLs
Macrophages phagocytose bone marrow RBC progenitors
Familial forms caused by decreased degranulation from CTLs and NK cells
Triggered by EBV infection

Elevated Ferritin and IL-2r
Anemia, Thrombocytopenia

Acute febrile illness
Hepatosplenomegaly (hepatitis)
Disseminated Intravascular Coagulation

Treat with immunosuppression and mild chemotherapy

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5
Q

White Cell Neoplasias
Etiologic Factors
Mutations (4) Viruses (3) Others (4)

A

Chromosomal translocations:
Help malignant cells proliferate and survive
Create oncoproteins useful for protection and growth
Proto-oncogenes made during receptor rearrangements

Human T cell Leukemia Virus 1 (HTLV-1)
EBV
HHV-8

Smoking (acute myeloid leukemia)
Iatrogenic (radiation/chemo)
Chronic Inflammation (H. pylori)
Inherited Genes

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6
Q

Leukemia vs Lymphoma Definitions

A

Leukemia
Neoplasms with widespread involvement of bone marrow and peripheral blood

Lymphoma
Start as discrete tissue masses

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7
Q

Principles of Lymphoid Neoplasms (6)

A

All daughter cells of malignant progenitor share same antigen receptor genes (immunophenotype)

Most cells will appear to be at some point of B/T cell differentiation

Often associated with immune abnormalities

Neoplastic B and T cells will act similar to non-neoplastic

Hodgkin Lymphoma spread orderly
Non-Hodgkin Lymphoma spreads widely and unpredictably

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8
Q

Acute Lymphoblastic Leukemia/Lymphoma
B Cell ALL: Population, Mutations (3), Origin Site
T Cell ALL: Population, Mutation, Origin Site
Prognosis (2) Immunophenotype (3) Lymphoblast Morphology (3)

A

B Cell Acute Leukemia:
Most common cancer of Children**
t(12;21) translocations, RUNX1, ETV6 mutations
Hypercellular Bone marrow: causes pancytopenia

T Cell Acute Lymphoma
Adolescent males
NOTCH1 mutations
Thymus

Aggressive in both cases
Excellent prognosis with aggressive chemotherapy

TdT (+)
CD10/CD19/CD20 (+) (B Cell ALL)
CD1-CD8 (+) (T Cell ALL)

Small amount of cytoplasm
No granules
Condensed Chromatin

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9
Q

Acute Lymphoblastic Leukemia
Clinical Features (6)
Favorable (5) and Unfavorable (3) Prognostics

A

Abrupt onset of symptoms within weeks:
Anemia, Bleeding
CNS symptoms
Mass Effects: bone pain, General Lymphadenopathy, Hepatosplenomegaly, Testicle Enlargement

Favorable: Age > 2, Low WBC, Hyperdiploidy, Trisomies, t(12;21)

Unfavorable: Age < 2 or adolescent/adult, Peripheral Blasts > 100,000, t(9;22) Philadelphia gene (BCR-ABL)

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10
Q

Chronic Lymphocytic Leukemia and Small LL

Population (2) Genetics (4) Origin, Immunophenotype (4) Clinical Features (5) Morphology (2)

A
  • CLL and SLL differ only in degree of lymphocytosis
  • CLL is >5000
Most common leukemia in Adults
Older Males (60s)

Deletions: 13q14.3, 11q, 17q
Trisomy 12q

Starts in Lymph Nodes

CD19
CD20
CD23
CD5

Asymptomatic at diagnosis
Small Ig spike present in some patients
Hypogammaglobulinemia 
Hemolytic anemia/Thrombocytopenia
Progression to Diffuse Large B cell Lymphoma (poor prognosis)

Proliferation Centers** (T cell aggregates)
Smudge cells

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11
Q

Follicular Lymphoma

Prognosis (2) Origin, Genetics (2) Morphology (3) Immunophenotype (6) Clinical Features (3)

A

Most common indolent non-hodgkin lymphoma
Incurable, survival 7-9 years

Starts in Lymph Nodes

t(14;18) IGH-BCL2 translocation**
MLL2 mutations

Centrocytes (small cleaved cells)
Centroblasts
Peritrabecular lymphoid aggregates in bone marrow

CD10, CD19, CD20
surface Ig
BCL6
BCL2*

Painless generalized lymphadenopathy
Waxing and waning course
Possible histologic transformation to Diffuse Large B cell lymphoma

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12
Q

Diffuse Large B Cell Lymphoma
Population, Pathogenesis (2) Morphology (2) Immunophenotype (3) Clinical Features (2) Prognosis (2)
Subtypes (2) with Descriptions (2/2)

A

Most common Non-Hodgkin Lymphoma*

Older males (median age 60)

BCL6 dysregulation
Or BCL2 rearrangements t(14;18)

Diffuse growth pattern
Large cells with round/oval nuclei, appear vesicular

CD19, CD20, surface Ig

Rapidly enlarging mass at nodal/extranodal sites
Waldeyer ring involvement

Aggressive, death if untreated
About half cured with aggressive chemo

Immunodeficiency-associated LBCL:
Associated with HIV
Caused by EBV infection

Primary Effusion Lymphoma:
Pleural/Ascitic effusion associated with HIV
Caused by KSHV/HHV-8

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13
Q

Burkitt Lymphoma
Populations (2) Origin, Genetics (2) Morphology (4) Immunophenotype (5) Prognosis (2)
Subtypes: Endemic (3), HIV-Associated, Sporadic (2)

A

Children
Young adults

Germinal centers

Translocation of c-MYC onto Chromosome 8

High mitotic index
Apoptotic cells
Phagocytes with clear cytoplasm (Starry sky pattern)
Blue cytoplasm with multiple nuclei (if in marrow)

CD10, CD19, CD20, IgM, BCL6

Aggressive, fastest growing tumor in humans
Curable with intense chemotherapy

Endemic: EBV associated, jaw mass, involves viscera
HIV-associated: aggressive
Sporadic: mass in illeocecum and peritoneum

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14
Q

Plasma Cell Neoplasms (Dyscrasias)

Most Common, Diagnostic Factors with Descriptions (2) Common Immunophenotype (2)

A

Multiple Myeloma

M Component in blood (monoclonal Ig)
Bence Jones Proteins (free light chains in urine)

CD138 (syndecan-1)
CD56

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15
Q

Multiple Myeloma

Pathogenesis (3) Morphology (5) Clinical Features (5) Diagnostic Features (3) Genetic Prognostics (4)

A

Rearrangements of the IgH locus - Cyclin D1 mutations
IL-6 keeps myeloma cells alive
MIP1a upregulates RANKL, causes bone resorption

Rouleaux Formation** (red cells stuck together)
Punched out bone lesions** 1-4 cm
Plasmablasts, Flame cells, Mott cells
Inclusions: Fibrils, Crystalline rods
Russell or Dutcher bodies (globular inclusions)

**Multiple Lytic bone lesions (Pathologic Fx)
Hypercalcemia (neuro Sx)
Renal failure (from Bence Jones)
Amyloidosis (from Bence Jones)
**Recurrent bacterial infections (main cause of death)

Bone marrow shows >30% atypical plasma cells
> 3g M proteins in blood (usually IgG or IgA)
> 6g Bence Jones proteins

Cyclin D1 translocations, good
13q, 17p t(4;14), bad

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16
Q

Solitary Myeloma (Plasmacytoma)
Description and Prognosis
Osseous and Extraosseous

A

Single bone lesion
Inevitably progresses to mutliple myeloma (10-20 years)

Extraosseous lesions often in upper respiratory tract
Cured via excision

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17
Q 
Smoldering Myeloma
Clinical Description (2) and Prognosis
A

Asymptomatic multiple myeloma-like syndrome
Serum M protein greater than 3g

Often progresses to MM over 15 years

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18
Q 
Monoclonal Gammopathy of Uncertain Significance
Clinical Description (2) and Prognosis
A

*Most common plasma cell disorder

Asymptomatic multiple myeloma-like syndrome
Serum M protein less than 3g

Per year ~1% of patients develop multiple myeloma

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19
Q

Lymphoplasmacytic Lymphoma
Population, Pathogenesis (3) Locations (4) Morphology (2) Immunophenotype (2) Clinical Features (4) Hyperviscosity Syndrome (4)

A

Presents in 60-70s

MYD88 gene mutations
Tumor cells undergo differentiation into plasma cells
Creates mostly monoclonal IgM

Starts in bone marrow
Moves to LNs, liver, spleen

PAS (+) Russell Bodies and Dutcher Bodies

CD20
Surface Ig (usually IgM)

Lymphadenopathy
Hepatosplenomegaly
Anemia
Autoimmune hemolysis by cold agglutinins

Waldenstrom Macroglobulinemia:
Visual impairment (from venous congestion)
Neurologic Problems
Bleeding
Raynaud’s Phenomenon (from cyroglobulinemia)

20
Q

Mantle Cell Lymphoma

Population (2) Genetics (3) Morphology (3) Immunophenotype (4) Clinical Features (2) Prognosis (3)

A

Males in 50s and 60s

t(11;14) translocation of Cyclin D1-IgH locus
Causes Cyclin D1 overexpression

Nodal tumor cells surrounds germinal centers
Homogenous population of small lymphocytes with irregular, deeply clefted nuclear contours
Lymphomatoid Polyposis (GI mucosa involvement)

Cyclin D1
CD19
CD20
CD5

Painless lymphadenopathy
Hepatosplenomegaly

Poor prognosis with no cure
Worst Prognosis: Blastoid variant and Proliferative expression

21
Q 
Marginal Zone Lymphomas
Pathologic Description (2), Common Features (3) Pathogenesis (3)
A

B cell lymphomas that arise in LN’s, spleen or extranodal tissues (MALTomas)
After spreading, can transform into diffuse large B cell lymphoma

Arise in areas of Chronic Inflammation
Remain localized for long periods
Regress if inciting inflammation eradicated (H. pylori)

Begins as polyclonal immune reaction
Acquired mutations allow cells to be Ag independent
Mutations upregulate BCL10 or MALT1

22
Q

Hairy Cell Leukemia

Population, Mutation, Morphology, Immunophenotype (4) Clinical Features (4) Prognosis

A

Middle aged (55) white males

Activating BRAF mutations

Cells with fine hair-like projections

CD11c
CD25
CD103
Annexin A1

Splenomegaly
Pancytopenia
Atypical mycobacterial infections
Bone marrow aspiration yields “dry tap”

Mostly indolent, excellent prognosis

23
Q

Peripheral T cell Lymphoma

Morphology (2) Immunophenotype (5) Clinical Features (5) Prognosis

A

Diffusely effaced lymph nodes
Pleomorphic mixture of malignant T cells

CD2, CD4, CD5, CD8
alpha-beta or gamma-delta T cell receptors

Generalized lymphadenopathy
Eosinophilia
Pruritis
Fever
Weight loss

Poor prognosis

24
Q

Anaplastic Large Cell Lymphoma

Mutation (2) Morphology (4) Immunophenotype (2) Prognosis

A

ALK gene rearrangements on Chr 2p23

Hallmark Cells: Anaplastic cells with horseshoe shaped nuclei and voluminous cytoplasm
Cells cluster around venules
Cells infiltrate lymphoid sinuses

ALK (+) cells are diagnostic
CD30

Aggressive but good prognosis

25
Q

Adult T cell Leukemia/Lymphoma

Pathogenesis (2) Morphology (2) Presentation (5) Prognosis (2)

A

Adults with Human T cell Leukemia Retrovirus-1 (HTLV1)
HTLV-1 encodes Tax protein that actives NFKB

Multilobated nuclei (cloverleaf/flower cells)
CD4+ T cells 
Skin lesions
Generalized lymphadenopathy
Hepatosplenomegaly
Peripheral blood lymphocytosis
Hypercalcemia

If only skin, indolent
If systemic, rapidly progressive, death within 1 year

26
Q

Mycosis Fungoides and Sezary Syndrome
Common Immunophenotype (4)
Mycosis: Phases (3) Histology (2) Late Phase (1)
Sezary: Presentation, Disease Association (2)

A

CD4+ T cells
CLA adhesion molecule
CCR4
CCR10

Mycosis Fungoides:
Phases: 1) Premycotic 2) Plaque 3) Tumor
Neoplastic T cells in Epidermis and Upper Dermis
Cerebriform appearnce to epidermis/dermis
Late phase spread to lymph nodes and bone marrow

Sezary Syndrome:
Generalized exfoliative erythroderma
Leukemia with Sezary cells showing cerebriform nuclei

27
Q

Large Granular Lymphocytic Leukemia

Mutation, Morphology (3) Immunophenotype (2) Clinical Features (2) Associated Syndrome (3)

A

STAT3 mutations

Either T cells or NK cells
Large lymphocytes with abundant blue cytoplasm
Coarse azurophilic granules

CD3 (T cells)
CD56 (NK cells)

Neutropenia
Anemia

Felty Syndrome: Rheumatoid arthritis, Splenomegaly, Neutropenia

28
Q

Extranodal NK/T cell Lymphoma

Pathogenesis, Morphology (3) Presentation (2) Prognosis

A

Highly associated with EBV**

Cell infiltrate surrounds small vessels, ischemic necrosis
Large azurophilic granules
Mostly NK cells, less T cells

Destructive nasopharyngeal mass**
Or mass in testes/skin

Aggressive, responds well to radiation (no chemo)

29
Q

Hodgkin Lymphomas

Populations (2) Origin/Spread (3) Pathogenesis (3) Prognosis

A

Most common cancer of teenagers
Usually presents in 30s

Arises in Lymph Nodes
Spreads to contiguous lymphoid tissues
Spreads to Spleen, then Liver, then Bone marrow

Activation of NFKB via:
EBV (cells express LMP-1)
IkB or A20 (TNFAIP3) mutations

Good prognosis with radiation therapy

30
Q 
Hodgkin Lymphomas
Characteristic Morphology (2) with Common Variants (3) Immunophenotype (3) Clinical Features (3)
A

Reed Sternberg Cells: Multinucleated giant cells with inclusion-like nucleoli
Variants: Mononuclear, Lacunar cells, Lymphohistiocytic

PAX5
CD15
CD30

Painless Lymphadenopathy
Fever, night sweats, weight loss (disseminated disease)
Cutaneous immune anergy

31
Q

Hodgkin Lymphoma Subtypes
Nodular Sclerosis Type
Pathogenenesis, Locations (2) and Morphology (3)

A

Most common

Not usually EBV associated

Starts in Lower Cervical and Supraclavicular LN’s

Lacunar cells
Few Reed Sternberg Cells
Deposition of collagen that divides up lymph nodes

32
Q

Hodgkin Lymphoma Subtypes
Mixed Cell Type
Population, Morphology, Clinical Feature, Prognosis

A

Older Males

Plentiful Reed Sternberg cells

Presents with Advanced tumor

Very good prognosis

33
Q 
Hodgkin Lymphoma Subtypes
Lymphocyte Rich Type
Cells Involved (3) Clinical Features (2)
A

Reactive Lymphocytes
Mononuclear cells
Reed Sternberg cells

Often EBV associated
Good Prognosis

34
Q

Hodgkin Lymphoma Subtypes
Lymphocyte Depletion Type
Populations (2) Cells Involved, Pathogenesis, Prognosis

A

Least common

Older adults
HIV (+) people

Reed Sternberg cells and their subtypes

EBV associated 90% of time

Least favorable prognosis

35
Q

Hodgkin Lymphoma Subtypes
Lymphocyte Predominance Type
Population (2) Morphology (3) Immunophenotype (2) Clinical Features (3)

A

*Non-Classical HL

Males under 35

Lymphocytic and Histiocytic variant cells
Multilobed nuclei (popcorn kernel cells)
Expanded B cell follicles (nodular growth)

CD20
BCL6

Cerivcal and Axillary lymphadenopathy
Can transform into Diffuse Large B cell lymphoma
NOT EBV associated

36
Q 
Acute Myeloid Leukemia
Major Subtypes (3) with Prognoses 
Genetic Aberrations (4) Pathogenesis (2) Causal Therapies (3)
A

AML with Genetic Aberrations: mostly good prognosis
AML with MDS-like Features: poor prognosis
Therapy-related AML: worst prognosis

t(8;21) RUNX1/ETO fusion*
inv(16) CBFB/MYH11 fusion*
t(15;17) RARA/PML fusion (best prognosis)
Karytotypic aberrations

Mutations of transcription factors of myeloid differentiation
Epigenetic alterations

After alkylator, radiation or anti-Topoisomerase II therapy

37
Q

Acute Myeloid Leukemia

Diagnosis, Morphology (3) Immunophenotype (2) Clinical Features (6)

A

Requires bone marrow biopsy showing at least 20% myeloid blasts

Myeloblasts with peroxidase (+) azurophilic granules
Auer rod granules
Monoblasts that are esterase (+)

CD33 and CD34

Anemia: fatigue
Neutropenia: fever
Thrombocytopenia: Bleeding, bruising, Petechiae
Opportunistic infections
Tissue Infiltration: Leukemia cutis (monocytic differentiation)
Granulocytic sarcoma

38
Q

Myelodysplastic syndromes

Etiologies (2) Mutations (4) Morphology (6) Clinical Features (3)

A

Primary - idiopathic
Secondary - genotoxic drugs or radiation

Similar epigenetic factors to AML
Mutations involving RNA splicing factors
Transcription factor mutations
TP53 mutations

Myeloblasts < 20%
Megaloblasts
Ring sideroblasts
Nuclear budding abnormalities: misshapen outlines
Pseudo Pelger Huet cells (bilobed neutrophils)
Pawn ball megakaryocytes

Mean age 70
Pancytopenia
Progression to AML

39
Q 
Myeloproliferative Disorders
Common Pathogenesis (2) Common Clinical Description (4)
A

Mutated, constitutively activated Tyrosine Kinases
Cause growth factor independence

Increased bone marrow proliferation
Increased extramedullary hematopoiesis
Spent phase: marrow fibrosis, peripheral cytopenias
Transformation to acute leukemia

40
Q

Chronic Myelogenous Leukemia

Population, Genetics (4) Origin, Morphology, Diagnosis (3) Clinical Features (4)

A

Adult males

Philadelphia Chromosome
(9;22)(q34;q11)
BCR-ABL fusion gene
Constitutively activated ABL tyrosin kinase

Originates from pluripotent HSC’s

Macrophages with wrinkled green-blue cytoplasm

Hypercellular marrow
Leukocytosis
Decreased Leukocyte Alkaline Phosphatase (LAP)

Insidious onset: mild anemia
Splenomegaly: abdominal fullness or pain
Hepatomegaly, lymphadenopathy
Blast crisis during late accelerated phase

41
Q

Polycythemia Vera

Mutation (2) Morphology (2) Labs (5) Presentation (5) Treatment

A

Activating JAK2 point mutation
Causes upregulated RBC production

Increased red cell, granulocyte and megakaryocyte precursors
Spent phase: marrow fibrosis

Low EPO
Elevated HCT
Increased Red Cell Mass (hyperviscous blood)
Leukocytosis
Thrombocytosis
Major bleeding episodes*
Venous thrombosis*
Hyperuricemia
Spent stage
May progress to AML

Treat with phlebotomy

42
Q

Essential Thrombocytosis

Mutations (2) Labs, Morphology, Diagnosis, Clinical Features (3) Treatment

A

Activating mutations in JAK2
Activating mutations in MPL

Isolated elevated platelet count (thrombocytosis)

Large platelets

Elevated megakaryocyte count on bone marrow biopsy

Erythromelalgia (burn/throb of hands/feet)
Bleeding
Thrombosis

Gentle chemo to stop thrombopoiesis

43
Q

Primary Myelofibrosis

Mutations (2) Pathogenesis (2) Clinical Features (2) Blood Smear (2) Prognosis (2)

A

Activating mutations in JAK2
Activating mutations in MPL

Obliterative bone marrow fibrosis via non-neoplastic fibroblasts making collagen
Caused by PDGF and TGF-Beta from megakaryocytes

Normochomic normocytic Anemia
Prominent splenomegaly

Tear drop shaped RBC’s (dacrocytes)*
Leukoerythroblastosis*

Poor prognosis, hard to treat
Death via infections, bleeding abnormalities

44
Q

Langerhans Cell Histiocytosis

Mutation, Common Morphology (2) Common Immunophenotype (2)

A

BRAF point mutation Val-Glu

Birbeck granules (pentalaminar tubules)
Abundant cytoplasm, nuclei with linear grooves

CCR6
CCR7

45
Q 
Multifocal Langerhans Cell Histiocytosis (Letterer-Siwe)
Clinical Features (7) Prognosis
A 
Seborrheic eruption cutaneous lesions
Chronic ottitis media
Mastoiditis
Hepatosplenomegaly
Lymphadenopathy
Lung lesions
Bone lesions

Rapidly fatal if untreated (50% survive if treated)

46
Q

Uni/Multifocal Unisystem Langerhans Cell Histiocytosis
(Eosinophilic Granuloma)
Morphology (2) Origin (3) Unifocal Clinical Description (2) Multifocal Clinical Description (5)

A

Langerhans cells mixed with eosinophils, lymphocytes, plasma cells, and neutrophils

Arises in medullary cavity of calvarium, ribs, femur

Unifocal painful bone lesions
Indolent, may resolve spontaneously or with excision

Multifocal erosive painful bone lesions
Presents in young children
Hand-Schuller-Christian Triad: Diabetes Insipidus, Exopthalmus, Calvarium defects

47
Q

Pulmonary Langerhans Cell Histiocytosis

Etiologies (2)

A

Chronic smoking

BRAF mutations

SBL Block 1 (6 decks)

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