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DISEASES of WHITE CELLS and LYMPHOID TISSUE

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Topics for Chapter 14 Leukopenia/Neutropenia

Leukocytosis

Lymphadenitis/Lymphadenopathy

(Malignant) Lymphoma

NON-Hodgkins Lymphoma

Hodgkins Lymphoma (Hodgkins Disease)

ALL/CLL (Acute/Chronic Lymphocytic Leukemia)

Multiple Myeloma

M1/M2/M3/M4/M5/M6/M7

Myeloproliferative Disorder

CML and Polycythemia Vera

Essential Thrombocytosis

Splenomegaly

Thymoma

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WBC/LYMPHOID DISORDERS

Review of Normal WBC Structure/Function

Benign Neutrophil and Lymphoid Disorders

Leukemias

Lymph Nodes

Spleen/Thymus REVIEW

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NEUTROPHILS

Normal TOTAL WBC count 6-11 K Neutrophils usually 2/3 of total normal

Myeloblast Promyelocyte Myelocyte

Metamyelocyte Band (stab) MatureNeutrophil (Poly, PMN, Neutrophilic Granulocyte)

Produced in red (hematopoetic) marrow, sequester

(pool) in spleen, live in peripheral blood, migrateOUT of vascular compartment PRN, live a coupledays normally

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NEUTROPHIL

Neutrophil

Polymorphonuclear Leukocyte,PMN, PML

Leukocyte

Granulocyte, Neutrophilicgranulocyte

Poly-

Polymorph

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NEUTROPHIL MATURATION

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LYSOSOMAL CONSTITUENTS PRIMARY Also called

AZUROPHILIC, orNON-specific

Myeloperoxidase Lysozyme (anit-Bact.)

Acid Hydrolases

SECONDARY Also called SPECIFIC

Lactoferrin (anti-Bact.)

Lysozyme (anti-Bact.) Alkaline Phosphatase

Collagenase

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FUNCTIONS

Margination Rolling

Adhesion

Transmigration (Diapedesis)

Chemotaxis

Phagocytosis:Recognition Engulfment Killing(digestion)

Equilibrium with splenic pool

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PELGER-HUET ANOMALY Genetic: Autosomal Dominant) Sometimes ACQUIRED (Pseudo-PELGER-HUET)

All neutrophils look like BANDS NOT serious, mostly a cute incidental finding

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CHEDIAK-HIGASHI SYNDROME Also genetic: Autosomal Recessive Abnormal LARGE irregular neutrophil granules Impaired lysosomal digestion of bacteria

Associated with pigment and bleeding disorders

CAN be serious, especially in kids

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LEUKO-penia/NEUTRO-penia

Neutropenia/Agranulocytosis INADEQUATE PRODUCTION

INCREASED DESTRUCTION

500-1000/mm3 is the DANGERzone!

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INADEQUATE PRODUCTION

Stem cell suppression, e.g., aplastic anemias DRUGS, esp. CHEMO, MANY antibiotics,

aminopyrene, thio-uracil, phenylbutazone

DNA suppression due tomegaloblastic/myelodysplastic states

Kostmann Syndrome: (A-R) (genetic, congenital)

Marrow usually shows granulocytic HYPO-plasia,just as in RBC and PLAT decreased production

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INCREASED DESTRUCTION

Immune mediatedBy itself (idiopathic), or as in SLEAfter sensitization by many drugs

Splenic sequestration, hypersplenism Increased peripheral demand, as in

overwhelming infections, esp. fungal Marrow usually shows granulocytic

HYPER-plasia, just as in RBC and PLATincreased destructions

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Leukocytosis/Neutrophilia

Marrow and splenic pool size Rate of release between pool and circulation

Marginating pool

Rate of WBCs (neutrophils/monocytes) leaving thevascular compartment

NON-vascular pools FIFTY times larger than thevascular pools

TNF/IL-1/cytokines stimulate T-cells to produceCSF, the WBC equivalent of EPO

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NEUTROPHIL INCREASES(e.g., NEUTROPHILIA)

BACTERIA

TISSUE NECROSIS, e.g., MI

DHLE BODIES and TOXICGRANULES are often seen withNEUTROPHILIA

Accompanied by a LEFT shift

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EOSINOPHIL INCREASES

(i.e., EOSINOPHILIA)

ALLERGIES (esp. DRUG allergies) PARASITES

Is there such a thing as eosino-penia?ANS: NO

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BASOPHIL INCREASES

(i.e., BASOPHILIA) RARE. VERY RARE. Period. But if you want to remember

something at least, remembermyeloproliferative diseases in

which ALL cell lines are increasedIs there such a thing as baso-penia?

ANS: NO

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MONOCYTE INCREASES

(i.e., MONOCYTOSIS)TB

SBE RICKETTSIAL DISEASES

MALARIA

SLE

IBD, i.e., ULCERATIVE COLITIS

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LYMPHOCYTE INCREASES

(i.e., LYMPHOCYTOSIS) TB VIRAL

Hep-A

CMV

EBV

Pertussis (whooping cough)

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MYELOPROLIFERATIVEdisorders

Also called chronic myeloproliferative disorders becausethey last for years

Differentiate: Myeloproliferative vs. Myelodysplastic

ALL marrow cell lines are affected, splenomegaly Proliferating cells do NOT suppress residual marrow

production, and go OUTSIDE marrow , and EXPANDmarrow to fatty appendicular marrow

Associated with EXTRA-medullary hematopoesis Chronic Myelogenous Leukemia (CML)

P. Vera

Essential Thrombasthenia (aka, Essential Thrombocytosis)

Myelofibrosis

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CML NOT AT ALL like an acute leukemia, but can

develop into an acute leukemia, as a conditioncalled a blast crisis

Age: adult, NOT kids

90% have the Philadelphia chromosome, which

are aberrations on chromosome #9 (BCR) and #22

(ABL), the BCR-ABL fusion

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CML Marrow 100% cellular, NOT 50% ALL cell lines increased, M:E ratio massively

increased, 50K-100K neutrophils with

SIGNIFICANT left shift, but not morethan 10% blasts

SIGNIFICANT SPLENOMEGALY!!!!! Significant breakthrough with BCR-ABL kinase

inhibitors!!! (90% remissions)

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Polycythemia Vera All cell lines increased, NOT just RBC

HIGH marrow cell turnover stimulatesincreased purines which often cause gout(10%)

BOTH thrombosis AND bleeding risks arepresent because the increased platelets are

AB-normal

Do not get blast crises, BUT can progress to

myelofibrosis

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ESSENTIAL THROMOCYTOSIS

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ESSENTIAL THROMOCYTOSIS

Platelet count often near 1 million/mm3 Often a diagnosis of exclusion. The RAREST of all myeloproliferative

disorders Giant platelets usually. Why? Ans: Quicker

release from marrow (RPW/RDW)(MPV/MCV)

Massively increased megakaryocytes in themarrow

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PRIMARY MYELOFIBROSIS

Rapid progressive marrow fibrosis Oldest age group of all the MPDs, >60

Can follow other MPDs. Why?

Usually the most extensive extramedullaryhematopoesis because the marrow is NOTthe primary site of hematopoesis

LEUKOERYTHROBLASTOSIS

Like CML, 10-20% can progress to AML

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WBC/LYMPHOID DISORDERS

Review of Normal WBC Structure/Function

Benign Neutrophil and Lymphoid Disorders

Leukemias

Lymph Nodes Spleen/Thymus

REVIEW

LEUKEMIAS

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LEUKEMIAS MALIGNANT PROLIFERATIONS of WHITE

BLOOD CALLS

In the case of neutrophilic precursors, the

primary process is marrow and peripheralblood, but can involve any organ or tissuewhich receives blood

In the case of lymphocytes, there is anintimate concurrence with malignantlymphomas

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Leukemias vs. Lymphomas All leukemias of lymphocytes have lymphoma

counterparts

Primary lymphomas can have leukemic phases,

including multiple myelomas

Any myeloid leukemia can infiltrate a lymph node, or anyother site, but if/when it does it is NOT called alymphoma, but simply a myeloid infiltrate INTO a lymphnode

ALL lymphomas are malignant proliferations oflymphocytes

ALL leukemias involve bone marrow changes

LYMPHOMAS

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LYMPHOMAS NODAL or EXTRANODAL

T or B

SMALL or LARGE CELLS

FOLLICULAR or DIFFUSE Hodgkins or NON-Hodgkins

F.A.B. classification is currently popular

this week (FrenchAmericaBritish), for theNON-Hodgkins lymphomas, also evolved into

the International classification

LEUKEMIAS

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LEUKEMIAS Acute or Chronic

Myeloid or Lymphocytic

Childhood or Adult

All involve marrow

All ACUTE leukemias suppress normalhematopoesis, i.e., have anemia,thrombocytopenia

Most have chromosomal aberrations

Some can respond DRASTICALLY to chemo, mostnotably ALL in children, even be cured!!!!

BLAST

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BLAST

WHITE CELL NEOPLASMS Leuk/Lymph

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Many have chromosomal translocations

Can arise in inherited and/or genetic diseases: Downs Syndrome (Trisomy 21)

Fanconis anemia (hereditary aplastic anemia)

Ataxia telangiectasia May have a STRONG viral relationship:

HTLV-1 (lymphoid tumors)

EBV (Burkitt Lymphoma) Human Herpesvirus-8 (B-Cell Lymphomas) (also KS)

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Can be caused by H. Pylori (gastric B-Celllymphomas)

Can follow celiac disease (glutensensitive enteropathy T-Celllymphomas)

Are common in HIV, B-Cell lymphomas,CNS lymphomas

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A.L.L./LYMPHOMAS* SUDDEN ONSET

ANEMIA, BLEEDING, FEVER Bone pain, adenopathy, hepatosplenomegaly

CNS: headaches, vomiting, nerve palsies

(* NB: These are pretty much the clinicalsymptoms of A.M.L. too and vice versa)

A L L /LYMPHOMAS

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A.L.L./LYMPHOMAS Lymphoblasts which can give rise either to T or B cells

are the cells of malignant proliferation

All lymphocytic leukemias CANNOT be classifiedindependently of lymphomas because they all have

lymphoma counterparts A.L.L. mostly in children

Most have chromosomal changes, hyperploidy,

Philadelphia chromosome, translocations SIGNIFICANT response to chemo: 90% remission, 75%

CURE!!!

A L L

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A.L.L.

C L L

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C.L.L. Unexplained sustained (months) lymph count of >

4000/mm3 is CLL, usually picked up on CBC M>F

Lymphs look normal and are NOT blasts No need for marrow exam for dx, but progressive

involvement of marrow, nodes, and other organs is the

usual biologic behavior Liver can be involved portally or sinusoidally

Translocations RARE, but trisomies and deletions

common

C

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C.L.L.

C L L

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C.L.L. HYPO-gammaglobulinemia 15% have antibodies against RBCs

or PLATS CANNOT be classified as separate

from lymphomas

MULTIPLE MYELOMA

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MULTIPLE MYELOMA DEFINED AS A MALIGNANT PROLIFERATION OF

PLASMA CELLS (i.e., former B-lymphocytes) Can have a leukemic phase, but the BONE

MARROW is the usual primary site of origin

Usually have MONOCLONAL GAMMOPATHIES Secrete Heavy and Light chains, and Light chains

in the urine is known as Bence-Jones protein

Usually have elevated IL-6 (bad prognosis)

PLASMA CELL l i f

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PLASMA CELL classic features OVAL cytoplasm, ROUND

nucleus off to side Cartwheel/Clockface

chromatin

Prominent Golgi or Hoff

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MONOCLONAL SPIKE on SPE

NORMAL MULTIPLE MYELOMA

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MULTIPLE MYELOMA BONE DESTRUCTION

Various deletions and translocations

Plasma cells usually 1-3% of marrow, but >20% or plasmacells in SHEETS is diagnostic

Plasma cells usually look normal

IgG >> IgA, other immunoglobulins are rare

Staph, Strep, E. coli infections

Bleeding* Amyloidosis

RENAL FAILURE

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Multiple Myeloma: Skull X-ray

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Solitary Plasmacytoma

Progression to MM is inevitable,

with time, perhaps 10-20 years even

M G U S

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M.G.U.S.

Monoclonal Gammopathy ofUnknownSignificance, i.e., no plasma cellproliferation is found

Age related

1% of 50-year olds, 3% of 70-year olds, etc.

Same chromosomal aberrations as MM, butgenerally follow a BENIGN course

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Other GAMMOPATHIES

Waldenstroms MACRO-globulinemia

IgM(associated with lymphomas) Heavy Chain Disease (associated with

lymphomas)

AMYLOID, follows MM and/or chronicgranulomatous diseases

A M L

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A.M.L. GENETIC ABERRATIONS INHIBIT DIFFERENTIATION

Many have various TRANSLOCATIONS

F.A.B. classifies them as M0 M7

MORE than 20% of BLASTS are needed in themarrow for a diagnosis of acute leukemia!!! (i.e.,

ANY kind of BLAST

NORMALLY, a marrow should have only about 1-2% blasts

A M L

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A.M.L. M0 Minimally differentiated

M1 AUER rods rare (COMMON)

M2 AUER rods common (COMMON) M3 Acute PRO-myelocytic leukemia

M4 AMML (myelo-Mono cytic) (COMMON) M5 Monocytic

M6 ErythroLeukemia

M7 Acute Megakaryocytic leukemia

NOTE: Diagnosis is CONFIRMED by special markers, not justvisual identification

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M0 M2

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M3

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M4-M5

AMML

Normal classic monocyte

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M6-M7

ERYTHROLEUKEMIA MEGAKARYOCYTIC LEUKEMIA

A M L

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A.M.L. Anemia

Thrombocytopenia (bleeding)

Petechiae Ecchymoses

Fever

Fatigue Lymphadenopathy

60% respond, BUT only 20 % are free of remission

after 5 years, WORSE than A.L.L.

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MYELO-DYSPLASTIC SYNDROMES

Increased risk of acute leukemias But, UNLIKE the myeloPROLIFERATIVE syndromes, NOT

a hypercellular marrow

Spontaneous or drug related (even > 5 yrs!)

Has marrow ABERRATIONS

REFRACTORY ANEMIAS

RINGED SIDEROBLASTS (Fe in mitochondria)

Nuclear BUDDING

EXCESS BLASTS, but LESS than 20%

About, say, 25% develop into acute leukemias

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Ring Sideroblasts and BUDS

LYMPH NODES

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LYMPH NODES Normal Structure, Function

Benign enlargement/Benign disease

Acute

Chronic (follicular vs. sinus histiocytosis)

Lymphomas/Malignant Lymphomas

Adjectives of various classifications

Features

STAGING

Metastatic disease TO lymph nodes

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CORTEX---SUB-capsular Sinus---Follicles (Pri? Or second.?)---PARA-follicular zoneMEDULLABlood flow?L m h flow?

Definition of TERMS

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Definition of TERMS Lymphadenopathy

Lymphadenitis

Dermatopathic

Normal size?

Palpation

What to do if a lymph node is enlarged?

Diffuse/Follicular T/B/NK, Small/Large, Cleaved/Non-cleaved

Precursor/Peripheral

HD/Non-HD

BENIGN ENLARGEMENT

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BENIGN ENLARGEMENT

Also called LYMPHADENITIS, and HYPERPLASIA

Can be ACUTE (tender), or CHRONIC (non-tender)

Usually SUBSIDE in, say, less than 6 weeks

FOLLICULAR HYPERPLASIA is enlargement of the cortical

secondary follicles and increase in number of the corticalsecondary follicles

SINUS HISTIOCYTOSIS is prominence in medullary sinuses(also called reticular hyperplasia)

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(MALIGNANT) LYMPHOMAS

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(MALIGNANT) LYMPHOMAS

Terms in historic classifications: Diffuse/Follicular, Small/Large, Cleaved/Non-cleaved

Hodgkins (REED-STERNBERG CELL) /NON-Hodgkins

Lukes, Rappaport, etc.

Working Formulation, WHO, NIH, FAB, Intl., etc.

B

T

PRECURSOR (less mature looking)

PERIPHERAL (more mature looking)

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DIFFUSE LYMPHOMA

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FOLLICULAR LYMPHOMA

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LARGE CELL LYMPHOMA

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SMALL CELL LYMPHOMA

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CLEAVED CELL LYMPHOMA

H i L h

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Hairy Lymphocyte

f

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FEATURES of LYMPHOMAS

The Antigen receptor genes re-arrangement PRECEDESmalignant transformation, so the cells areMONOCLONAL, NOT the usual POLYCLONAL

85% B-cell, 15% T-Cell

The tumor cells congregate wherever T and B cellcongregate normally however

DISRUPTED or EFFACED normal architecture,

obliterated subcapsular sinus HD/Non-HD staging CRUCIALLY IMPORTANT, esp. HD.

Why? HD grows (spreads) more linearly, i.e., more

predictably.

LATEST CLASSIFICATION

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LATEST CLASSIFICATION

NON-HODGKINPRECURSOR B

PERIPHERAL BPRECURSOR T

PERIPHERAL T

HODGKINS DISEASE (i.e., HODGKINS

LYMPHOMA) NS, LP, MC, LD

PRECURSOR B

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PRECURSOR B Precursor B LYMPHOBLASTIC

LEUKEMIA/LYMPHOMA

PERIPHERAL B

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PERIPHERAL B CHRONIC LYMPHOCYTIC LEUKEMIA/LYMPHOMA

B-Cell PRO-lymphocytic LEUKEMIA

Lymphoplasmacytic

Splenic and Nodal Marginal Zone

EXTRA-nodal Marginal Zone

Mantle Cell Follicular

Marginal Zone

Hairy Cell Leukemia Plasmacytoma/Multiple Myeloma

Diffuse B Cell

BURKITT LYMPHOMA (Starry Sky)

PRECURSOR T

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PRECURSOR T Precursor T LYMPHOBLASTIC

LEUKEMIA/LYMPHOMA

PERIPHERAL T and NK

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PERIPHERAL T and NK T-Cell PRO-Lymphocytic Leukemia

Large Granular

Mycossis fungoides/Sezary Cell syndrome (skin)

Peripheral T-Cell

Anaplastic large cell

Angioimmunoblastic T-Cell

Enteropathy-associated T-Cell

Panniculitis-like

Hepatosplenic gamma-delta Adult T-Cell

NK/T Cell nasal

NK-Cell leukemia

LYMPHOCYTE MARKERS (CD-)

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i.e., LYMPHOCYTE ANTIGENS T-Cell: 1,3,4,5,8

B-Cell: 10 (CALLA), 19,20,21,23,79a

Mono/Mac: 11c, 13, 14, 15, 33, 34

STEM: 34

RS: 15, 30

All: 45 (Leukocyte Common Antigen) NK: (16, 56)

HODGKINS DISEASE

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HODGKINS DISEASE NEED R-S (Reed-Sternberg, or Sternberg-Reed)

cells for correct diagnosis

NODULAR SCLEROSIS (Young Women), theR-S cells may be called LACUNAR cells

MIXED CELLULARITY

Lymphocyte RICH Lymphocyte POOR

Lymphocyte PREDOMINANCE

STERNBERG REED CELL

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STERNBERG-REED CELL

STAGING, HD & NHD

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STAGING, HD & NHD I ONE NODE or NODE GROUP

II MORE than ONE, but on ONE side of diaph.

III BOTH sides of diaph., but still in nodes only

IV OUTSIDE of NODES, e.g., liver, marrow, etc.

A No systemic symptoms

B fever and/or night sweats and/or 10% weight loss

METASTATIC CARCINOMA

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METASTATIC CARCINOMA

Perhaps the single most important staging andprognostic feature of tumors

The metastatic cells FIRST enter into theSUBCAPSULAR SINUS

The tumor may replace the entire node andenlarge it

The tumor may be focal

The tumor usually looks the same as its primary

or other metastases

The tumor usually ENLARGES the node

METASTATIC SQUAMOUS CELL

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QCARCINOMA

METASTATIC ADENOCARCINOMA

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METASTATIC ADENOCARCINOMA

SUBCAPSULAR SINUS

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SUBCAPSULAR SINUS

SPLEEN

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SPLEEN 150 grams POST-LUQ (just like kidney, 1/10 of liver)

Bordered by diaphragm, kidney, pancreas, splenic flexure,stomach

SMOOTH & GLISTENING capsule

50% RED pulp, 50% WHITE pulp

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ABNORMAL SPLEEN

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ABNORMAL SPLEEN

ABNORMAL SPLEEN

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ABNORMAL SPLEEN

SPLENIC FUNCTION

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SPLENIC FUNCTION

REMOVE OLD BLOOD CELLS

MAJOR SECONDARY ORGAN of the IMMUNESYSTEM

HEMATOPOIESIS

SEQUESTER (POOL) BLOOD CELLS 15% of bodys PHAGOCYTIC activity is in the

spleen (liver has >80)

SPLENOMEGALY

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SPLENOMEGALY CONGESTIVE vs INFILTRATIVE HYPERSPLENISM

AnemiaLeukopenia

Thrombocytopenia DECISION for SPLENECTOMY

SPLENOMEGALY

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SPLENOMEGALY INFECTIONS: TB, Mono, Malaria, Fungus

PORTAL HTN: CHF, CIRRHOSIS, PV Thromb.

LYMPHOHEMATOGENOUS: Leuk, Lymph, esp. CML

IMMUNE: RA, SLE STORAGE: Gaucher, Niemann-Pick

MISC: Amyloid, mets (melanoma, lymphoma, Germ celltumors of testis)

LONG STANDING CONGESTION breeds FIBROSIS

INFARCT

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INFARCT

PRIMARY TUMORS (RARE)

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PRIMARY TUMORS (RARE)

HEMANGIOMA

LYMPHANGIOMA fibroma

osteoma

chondroma

LYMPHOMA

MISC

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MISC Congenital Absence (very rare)

Accessory spleens (very very

common, especially withsplenomegaly!)

RUPTURE

THYMUS

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THYMUS Mother of all T-Cells

Massive in newborns, virtually absent in theelderly, bilobed

Under manubrium 1) Thymocytes

2) Epithelial Ret. Cells

3) Hassals Corpuscles

HASSALs CORPUSCLES

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HASSAL s CORPUSCLES

DISEASES

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DISEASESHYPOPLASIA/APLASIA

DiGeorge Syndrome

CYSTS (incidental)

THYMOMAS

THYMOMAS

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THYMOMAS

ALL (most) thymomas show counterparts ofBOTH lymphoid as well as epithelial reticularcells, hence, the classic name

LYMPHOEPITHELIOMA Benign thymoma: (encapsulated)

Malignant Thymoma I: (locally invasive)

Malignant Thymoma II: (easily metastasizable)

THYMOMAS

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THYMOMAS

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