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Flashcards in Leukocyte disorders wk7t10 Deck (18):
1

Normal WBC counts

PMN

Lymph

mono

eosino

baso

WBC count- 4,000-10,000 

PMN- 50-70%

lymph-20-40%

mono- 1-6%

eosino 1-5%

baso- 0-2%

2

WBC disorders overview

- too many (leukocytosis)

reactive (infection) vs. neoplastic (leukemias, lypmphomas)

- dysfunction

congenital, toxic, neoplastic

- too few (leukopenia)

decreased production vs increased destruction vs sequestration

3

Neutropenia

- symotoms related to infection, typically bacterial and or fungal

- neutrophil count < 1000

- etiology: decreased or ineffective productiion vs increased destruction, splenic squestration

4

Aplastic anemia

overview

- a disease which results from decreased marrow production of blood cells

- failure of normal hematopoiesis

- usually pancytopenia- involving all 3 cell lines (myloid, erythroid, megakaryocytic)

5

Aplastic anemia- clinical findings

- signs and symptoms

- lab findings

-signs and symptoms

those of pancytopenia: weakness, pallor

infection, fever, bleeding paticheae

- lab findings

pancytopenia (neutropenia, anemia, thrombocytopenia)

bone marrow is hypocellular with decreased or absent myeloid, erythroid and megokaryocytic

 

6

aplastic anemia

etiology

- most cases idiopathic

- can be due to:

drugs, toxins

infections (hep)

radiation exposure

immune mediated

other lconal diseases

7

Aplastic anemia

- prognosis and therapy

Variable severity depending on the degree of cytopenias

- severe

pmn < 500, retic <1% ptl<20,000 marrow<20% cellularity

infections, bleeding, rbc tx dependent

90% die in 1 yr 

- recommended therapies

transfusion, antibiotics, growth factors, immune suppression (atg), allogenic hematopoietic stem cell transplant

 

8

Lymphoid disorders

- Tcells, Bcells, plasma cells, NK cells

- lymphoproliferative disorders: abnormal production or accumulation of lymphoid cells with clinical behavior reminiscent of the ontogeny of cells

- plasma cell dyscrasias: neoplastic and preneoplastic disorders of plasma cells

 

9

Leukocytosis

overview, how to figure out source

Identify cell line is increased

- neutrophilm lymphocyte, monocyte, eosinophil, basophil

identify stage of maturation of the cells

- mature vs. immature blasts

identify the etiology

- a primary marrow abnormality

(neoplastic of preneoplastic)

- secondary (appropriate marrow response to external signals. ie. infection)

10

Lymphocytosis

causes

absolute lymphocytes > 5000

acute infections: 

pertussis, EBV, CMV, hep, other viral

chronic infection:

brucellosis, TB, syphilis

Neoplastic

chronic lymphocytic leukemia

acute lymphocytic leukemia

Lymphomas

11

Ontogeny of B cells when considering lymphomas

- immature B cell- Ig rearrangment, tDt expression

= acute lymphoblastic leukemia

- activated B cell in germinal ceter

= diffuse large B cell nonHadgkin's lymphoma and hodgkins disease

- AB secreting plasma cell, Memory Bcell

= chronic lymphocytic leukemia

12

Chronic lymphocytic leukemia

(CLL)

lymphoproliferative disorder with:

- lymphocytosis, lymphadenopathy, organomegally

- usyally monclonal mature Bcells

30% of all leukemias in US

13

CLL clinical staging

staging

0= lymphocytosis of blood and marrow

1= lymphocytosis + lymphadenopathy

2= lymphocytosis + spplenomegaly +/or hepatomegaly

3=lymphocytosis + anemia (hg<11)

4= lymphocytosis + thrombocytopenia (plt <100K)

 

14

CLL clinical features

lymphocytosis

lymphadenopathy

hepatosplenomegaly

frequent infections

immunologic abnormalities

histologic transfromation

secondary malignancies

15

CLL lab findings

lymphocytosis with morphologically mature B cells

MONOCLONAL LYMPHOCYTES

Coexpression of CD5 (T cell marker) with CD19 & 20

Anemia, thrombocytopenia

hypogammaglobulinemia

marrow infiltration with cll cells

lymph nodes (diffuse small cell lymphoma)

16

CLL treatment and prognosis

responsive but not curative standard rx

Treat only if symptomatic

- alkylators, fludarabine, combo chemo, STEROIDS, monclonal AB therapy, treat infections

- High dose therapy with blood or marrow transplant in younger patient with allo- donor

-

17

hairy Cell leukemia

lymphoproliferative disorder with abnormal blood and marrow lymphocytes with fine filamentour "hairy" projections

Usually Bcells. Stain for: tartrate resistant acid phosphatase "trap", monoclonal surface immunoglobulins and Fc receptors.

pancytopenia, splenomegaly, infections, immune abnormalities

Responsive to deoxycoformycin, alpha interferen, splenectomy

18

Chronic Tcell luekemias and lymphomas

Mycosis fungoides/Sezary’s syndrome
- Cutaneous T cell lymphomas
- Circulating Sezary cells

Large granular Lymphocytosis syndrome
- T/NK cell disorder
- Frequent pancytopenia

Adult t cell leukemia/lymphoma
- HTLV-1 associated
- Lymphocytosis, lymphadenopathy,
- hypercalcemia, lytic bone lesions