Leukocyte disorders wk7t10

Question 1

Normal WBC counts

PMN

Lymph

mono

eosino

baso

Answer 1

WBC count- 4,000-10,000

PMN- 50-70%

lymph-20-40%

mono- 1-6%

eosino 1-5%

baso- 0-2%

Question 2

WBC disorders overview

Answer 2

- too many (leukocytosis)

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

- dysfunction

congenital, toxic, neoplastic

- too few (leukopenia)

decreased production vs increased destruction vs sequestration

Question 3

Neutropenia

Answer 3

• symotoms related to infection, typically bacterial and or fungal
• neutrophil count < 1000
• etiology: decreased or ineffective productiion vs increased destruction, splenic squestration

Question 4

Aplastic anemia

overview

Answer 4

• 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)

Question 5

Aplastic anemia- clinical findings

• signs and symptoms
• lab findings

Answer 5

-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

Question 6

aplastic anemia

etiology

Answer 6

• most cases idiopathic
• can be due to:

drugs, toxins

infections (hep)

radiation exposure

immune mediated

other lconal diseases

Question 7

Aplastic anemia

• prognosis and therapy

Answer 7

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

Question 8

Lymphoid disorders

Answer 8

• 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

Question 9

Leukocytosis

overview, how to figure out source

Answer 9

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)

Question 10

Lymphocytosis

causes

Answer 10

absolute lymphocytes > 5000

**acute infections: **

pertussis, EBV, CMV, hep, other viral

chronic infection:

brucellosis, TB, syphilis

Neoplastic

chronic lymphocytic leukemia

acute lymphocytic leukemia

Lymphomas

Question 11

Ontogeny of B cells when considering lymphomas

Answer 11

- 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

Question 12

Chronic lymphocytic leukemia

(CLL)

Answer 12

lymphoproliferative disorder with:

• lymphocytosis, lymphadenopathy, organomegally
• usyally monclonal mature Bcells

30% of all leukemias in US

Question 13

CLL clinical staging

Answer 13

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)

Question 14

CLL clinical features

Answer 14

lymphocytosis

lymphadenopathy

hepatosplenomegaly

frequent infections

immunologic abnormalities

histologic transfromation

secondary malignancies

Question 15

CLL lab findings

Answer 15

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)

Question 16

CLL treatment and prognosis

Answer 16

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

-

Question 17

hairy Cell leukemia

Answer 17

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

Question 18

Chronic Tcell luekemias and lymphomas

Answer 18

• *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