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White blood cell disorders Flashcards

1
Q

What is a normal WBC count? What are some cuases of neurtropenia? Treatment? What are some causes of lymphopenia? What are some causes of neutrophillic leukocytosis? Monocytosis? Eosinophilia? Bsophilia? Lymphocytic leukocytosis?

A

5-10 K

Neutro=Drug toxicity and severe infection
GM-CSF or G-CSF

Lympho: Immunodeficiency, high cortisol state, autoimmne destruction, and whole body radiation

Neutro cytosis: Bacterial infection or tissue necrosis (left shift), high cortisol state (impairs adhesion)

Mono: chronic inflammatory states and malignancy

Eosinophilia: Allergic reactions, parasitic infections and hodgkins lymph

Basophilia: chronic myeloid leukemia

Lymph cytosis: viral infections, bordetella pertussis infections (can’t leave LN)

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2
Q

How is EBV transmitted? What does it primaryilly infect? What are the results of that infection? What does the monospot test detect? Definitive diagnosis? Complications?

A

Saliva (kissing disease)

B cells, Liver (HM, elev. liver enzymes), oropharynx

CD8+ T cell response—>LAD, splenomegaly, high WBC cont with atypical lymphocytes

Detects IgM Abs that cross react with horse or sheep RBCs. Positive within 1 week (only with EBV, not CMV)

Serological testing of EBV viral capsid

Splenic rupture (no contact sports)
Rash with ampicillin
incr. risk for recurrence and B cell lymphoma

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3
Q

What is acute leukemia? How is it defined? What is a typical presentation? Pathophys? Lab findings? How is it divided?

A

Neoplastic prolif. of blasts; >20% blasts in bone marrow.

They crowd out normal hematopoesis leading to anemia (fatigue), thrombocytopenia (bleeding), or neutropenia (infection)

Blasts (large, immature cells with punched out nuclei) leave BM leading to high WBC count

Acute lymphoblastic leukemia and acute myelogenous leukemia

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4
Q

What is acute lymphoblastic leukemia? How are lymphoblasts characterized? What are the 2 types? Age? ASsociations?

A

Lymphoblasts (>20%) in bm

Positive nuclear staining for TdT, a dna polymerase (not positive in myeloblasts or lymphocytes

Children, DS (after age 5)

B-ALL, T-ALL

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5
Q

What is the most common type of ALL? What are its markers? What is the treatment (response)? Where can it spread to? What are two mutations associated with it and what is the prognosis for each? Which is less common? Markers? Presentation? Another name for it?

A

B-ALL

TdT+, CD10, 19, 20

Responds to chemo (first to scrotum and CSF=can spread there)

t(12:21) good prognosis, seen in children
t(9:22) philadelphia chromosome: adults

T-ALL

Tdt+, CD2-8, no CD10

Teenagers-mediastinal mass (thymic) (also called acute lymphoblastic lymphoma for this reason

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6
Q

What is AML? How are they characterized? Age? Mutations of acute promyelocytic leukemia? Pathophys? Treatment? Pathophys of acute megakaryoblastic leukemia? Association? Pathophys of acute monocytic leukemia? What cell marker is lacking? Where does it infiltrate?

A

Acute myeloid leukemia

Positive cytoplasmia staining for MPO (crystal aggregates are called Auer Rods)

APL: t(15:17), retinoic acid receptor to chrom. 15, blocks maturation leading to blasts.
Abnormal promyelocytes contain granules that incr. risk for DIC
Treatment is with all trans retinoic acid

Acute monocytic: monoblasts (no MPO), infiltrate gums

Acute megakaryo: lack MPO, down syndrome

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7
Q

What can lead to AML? How does this disorder present? How does it lead to AML? What else can it lead to? How?

A

Preexisting dysplasia (myelodysplastic syndromes)

cytopenias, hypercellular BM, incr. blasts (<20%)

Akylating agents or chemotherapy make conversion more likely

death from Infeciton or bleeding

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8
Q

What is chronic leukemia? How is it characterized? How is the onset? Age? 4 types?

A

Neoplast. prolif. of mature circ. lymphocytes; high WBC count.

Insidious in onset; older adults

Chronic lymphocytic leukemia
Hairy cell leukemia
Adult T cell leukemia/llympoma
Mycosis fungoides

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9
Q

What is CLL? Cell markers? Blood smear? What else can it be called? Why? Complications?

A

Chronic lymphocytic leukemia

Naive B cells (CD5 and CD20)

Incr. lymphocytes and smudge cells on blood smear

Small lymphocytic lymphoma; can lead to LAD

Hypogammaglobulinemia (infection)
Autoimmune hemolytic anemia
Transformation to large B cell lymphoma marked by enlarging LN or spleen

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10
Q

What is hairy cell leukemia? What are the cells like? markers? Positive and negative clinical features? Treatment? Mechanism?

A

Mature B cells with hairy cytoplasmic processes

Tartrate resistant acid phosphatase (TRAP)

Splenomegaly and dry tap on bone marrow aspiration (fibrosis)

2;CDA (cladribine) ADA inhibitor

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11
Q

What is ATLL? Assocations? locations? Clinical features?

A

Adult T cell leuk/lymph

mature CD4+ T cells

HTLV-1; japan, caribbean

Rash, LAD with HSM, lytic punch out bone lesions with hypercalcemia

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12
Q

What is mycosis fungoides? Clinical features? What is sezary syndrome? Smear?

A

Mature CD4+ t cells that infiltrate skin

localized skin rash, plaques, nodules, pautrier microabscesses (aggregates of neoplasm)

Sezary: spread to blood, cerebriform nuclei (sezary cells) on smear

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13
Q

What are myeloproliferative disorders? Age? Lab findings? What is proliferated? how are they characterized? complications?

A

Neoplastic prolif. of mature cells of myeloid lineage; late adulthood

All myeloid lineages are incr. but syndromes are characterized based on which is most prominent

Hyperuricemia and gout (high turnover)
Marrow fibrosis
Transformation to acute leukemia

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14
Q

What is chronic myeloid leukemia? What line is especially increased? What cell is characteristically increased? What is the mutation? Treatment? What is a common clinical finding? What does it mean?

A

CML

Mature myeloid cells; granulocytes; basophils

t(9;22), BRC-ABL fusion, incr. tyrosine kinase activ.
Imatinib (blocks tyrosine kinase)

splenomegaly: soon progresses to AML or ALL

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15
Q

What is a leukemoid reaction and how is it distinguished from CML?

A

Acute inflammatory response to infection.

Incr. WBC dounts with incr. neutrophils and neutrophil precursors (band cells)=Just like CML.

CML: Negative leukocyte alk phos stain (LAP)
Incr. basophils
t (9;22)

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16
Q

What is polycythemia vera? What cells are specifically incr. What else? Mutation? Clinical symptoms? Treatments? Prognosis? How can it be distinguished from reactive polycythemia?

A

Myeloid cells, especially RBCs. Aslo granulo. and platelets

JAK2 kinase

Hyperviscosity:
blurry vision and headache
Incr. risk of venous thromb
flushed face
Itching after bathing (incr. mast cells)

Phlebotomy, hydroxyurea

Death in one year w/o treatment

Reactive: EPO levels incr. (decr. in PV) with decr. SaO2 (normal in PV). If it is caused by ectopic EPO production from RCC, EPO is high, SaO2 normal.

17
Q

What is essential thrombocythemia? Cell primarily increased? Others? Mutation? Symptoms?

A

Mature myeloid cells, platelets; also rBCs and granuloctyes.

JAK2 kinase

incr. bleeding and/or thrombosis
Rarely progresses to marrow fibrosis or acute leukemia
No significant risk for hyperuricemia or gout

18
Q

What is myelofibrosis? Primary cells? Mutation? Pathophys? Symptoms? Smear?

A

Mature myeloid, especially megakaryocytes

JAK 2 kinase

Excess PDGF causing marrow fibrosis

Splenomegaly (extramedullary hematopoesis
Infection, thrombosis, bleeding

Smear: tear drop RBCs, nucl. RBCs, and immature granulocytes

19
Q

What is LAD? When is it painful? Painless? What cauess follicular hyperplasia? Paracortex hyperplasia? Hyperplasia of sinus histiocytes?

A

Painful: acute infection

Painless: chronic inflammation, metastatic CA Lymphoma

follicular: RA and early HIV
paracortex: Viral infections
sinus: LNs draining a tissue with cancer

20
Q

What is a lymphoma? Where does it arise? What are the two types? How are they further divided? Which lymphomas are in each category?

A

Neoplastic prolif. of lyphoid cells that forms a mass; LN or extranodal

non-hodgkin lymphoma (60%) and Hodgkin

NHL classified by cell size, pattern of cell growth, surface markers, and cytogenic translocations

small B cells: follicular lymphoma, mantel cell lymph, marginal zone, and small lymphocytic

Intermediate sized: burkitt

large b cells: diffuse large b cell lymphoma

21
Q

What prolif in follicular lymphoma? surface marker? What is the pattern of proliferation? Age? Presentation? Mutation? Pathophys? Who is treated and how? Complication/presentation? How is it distinguished from reactive follicular hyperplasia?

A

CD20+ small b cells; follicle like nodules

Late adulthood: painless LAD

t(14;18) BLC2 on 18 to Ig heavy chain on 14
Overexpression of Bcl2–>antiapoptosis

symptomatic treated with low dose chemo and rituximab (anti CD20)

Diffuse large B cell lymphoma; enlarging LN

Disruption of normal LN architecture, lack of tingible body macrophages in germinal center, bcl2, monoclonality

22
Q

What prolif. in mantle cell lymphoma? presentation? age? Mutation? Pathophys?

A

Small b cells (CD20) that expands mantle zone

Late adulthood; painless LAD

t(11;14): cylclin D1 gene to Ig heavy chain locus.
Promotes overexpression leading to g1/s transition

23
Q

What prolif. in marginal zone lymphoma? What is it associated with? What forms marginal zone? What is a MALToma?

A

small b cells (CD20).

hashimotos, sjogrens, and h pylori gastritis

post germinal center b cells

marginal zone lymphoma in mucosal sites

24
Q

What prolif. in burkitts lymphoma? Association? Presentation? Age? Clinical manifestations in african form? sporadic form? Mutation? pathophys? How is it characterized?

A

intermediate size b cell (CD20)

EBV

Children to young adult; extranodal mass
Africa: Jaw
Sporadic: abdomen

t(8;14) c-myc to ig heavy chain
c-myc oncogene promotes cell growth

High mitotic index and starry sky appearance on microscopy

25
Q

What prolif. in diffuse large b cell lymphooma? How do they grow? presentation? age?

A

large B cells (CD20); grow diffusely in sheets

clinically aggressive

sporadically or from low grade lymphoma

late adulthood; enlarging LN or extranodal mass

26
Q

What is hodgkins lymphoma? Cells? Describe the cells? Cell marker? Pathophys/results? What make up the bulk of the tumor? 4 subtypes? Most common? Classic presentaiton of most common? Best prognosis? What is mixed cellularity associated with? Which is most aggressive?

A

Reed sternberg cells; large b cells with multilobed nuclei and prominent nucleoli (owl eyed nuclei).

CD15 and CD30

RS cells secrete cytokines:
B symptoms (fever, chills, weight loss, and night sweats)
attract reactive lymphocytes, etc.
Fibrosis

Nodular sclerosis, lymphocyte rich, lympocyte depleted, mixed cellularity

Nodular most common type: Enlarging cervical or mediastinal LN in a yougn adult, usually female
Bands of sclerosis; RS cells in lake like spaces

Rich has best prognosis

Mixed=eosinophils

depleted is most aggressive; HIV and elderly`

27
Q

What is multiple myeloma? Serum marker? Clinical features? Pathophys? Most common cause of death? What is MGUS? How does it differ from multiple myeloma? What can it ead to? Age/presentation?

A

malignant prolif of plasma cells in BM

High serum IL-6

Bone pain with hypercalcemia; activates RANK; lytic punched out lesion (vertebrae and skull); incr. risk for fracture

M spike on SPEP (IgG or IgA)

Infection; most common cause of death

rouleaux formation of RBCs

Primary AL amyloidosis

Proteinuria (bence jones protein)

monoclonal gammopathy of undetermined significance
Incr. serum protein with M spike but no other features of MM.
Found in elderly; can lead to MM

28
Q

What is waldenstrom macroglobulinemia? Clinical features? Treatment for acute complications?

A

B cell lymphoma with monoclonal IgM production

Generalized LAD (no lytic bone lesions)

M spike (IgM)

Visual and neuro deficits-IgM hyperviscosity

Bleeding-hyperviscosity leads to defective platelet aggregation

Plasmapheresis

29
Q

What is langerhans cell histocytosis? What is seen on electron microscopy? Immunohistochemistry markers? What rre the 3 kinds? How do they compare in proliferation, presentation, age, and biopsy?

A

Neoplastic prolif. of langerhans cells (DC cells in skin; derived from BM monocytes; present antigen to t cells)

Birbeck granules (tennis racket)  
CD1a+ and S100+

Letterer-Siwe
Malignant proliferation
Skin rash and cystic skeletal defects in infants
multiple organs involved; rapidly fatal

Eosinophilic granuloma
benign prolif in bone
pathologic fracture in adolescent; no skin
biopsy: langerhans w/ mixed inflammatory cells

hand-schuller-christian
malignat prolif.
scalp rash, lytic skull defects, diabetes insipidus, and exopthalmos in a child.

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