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63 Cards in this Set

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CML
MYELOFIBROSIS
LEUKEMOID REACTION
anemia?
+/-
YES
+/-
CML
MYELOFIBROSIS
LEUKEMOID REACTION

Teardrop RBCs?
NO
YES
NO
CML
MYELOFIBROSIS
LEUKEMOID REACTION

Nucleated RBC’s?
NO
YES
NO
CML
MYELOFIBROSIS
LEUKEMOID REACTION

WBC>100,000
OFTEN
VERY RARE
RARE
CML
MYELOFIBROSIS
LEUKEMOID REACTION

WBC 50-100,000?
COMMON
RARE
COMMON
CML
MYELOFIBROSIS
LEUKEMOID REACTION

WBC 30-50,000?
COMMON
UNCOMMON
COMMON
CML
MYELOFIBROSIS
LEUKEMOID REACTION

Basophils increased?
YES
NO
NO
CML
MYELOFIBROSIS
LEUKEMOID REACTION

Eosinophils increased?
YES
NO
NO
CML
MYELOFIBROSIS
LEUKEMOID REACTION

>5% Myelocytes or younger cells?
YES
MAYBE
NO
CML
MYELOFIBROSIS
LEUKEMOID REACTION

LAP?
LOW
VARIABLE
HIGH
CML
MYELOFIBROSIS
LEUKEMOID REACTION
Marrow?
MYELOID HYPERPLASIA & BASOS & EOS

FIBROSIS

MYELOID HYPERLASIA
CML
MYELOFIBROSIS
LEUKEMOID REACTION

Ph1 chromosome
KEY TO DX
NO
NO
Normal neutrophil range
1800-6700
Normal Lymphocyte range
1400-3900
Pelger-Huet anomaly
neutrophils are generally bilobed and may be unsegmented
May-Hegglin anomaly
basophilic inclusions in WBC’s, associated with thrombocytopenia
Chediak-Higashi syndrome
giant neutrophil granules (abnormal killing of bacteria in lysosomes
Alder’s anomaly
deep purple granules. May have no clinical association or may be associated with mucopolysaccharide disorders.
Maroteaux-Lamy syndrome
abnormal red-violet granules and lymphocyte vacuoles

associated with mucopolysaccharide disorders
which leukemia is 85% of adult acute leukemia
ACUTE NON-LYMPHOCYTIC LEUKEMIA
presentation of Acute Non Lymphocytic Leukemia (AML)
Can present either as pancytopenia, or with anemia, thrombocytopenia, and elevated WBC due to the presence of myeloblasts in the peripheral blood.
Which leukemia is 85% of childhood acute leukemia
ACUTE LYMPHOBLASTIC LEUKEMIA
DIFFERENTIAL DIAGNOSIS OF AN ELEVATED GRANULOCYTE COUNT (5)
1 Infection – mild elevation of WBC, 10,000 to 15,000. Almost all polys, some bands

2 Chronic myelogenous leukemia – WBC may be very high, >50,000 or even > 100,000. At least 5% myelocytes or younger

3 Myelofibrosis - usually normal or low WBC, but may look like CML, w/out phili chrom

4 Leukamoid reaction - exaggerated response to infection. WBC>30,000 or 40,000 is generally considered a leukemoid reaction.

5 - P Vera – an elevated WBC may be present though elevated hematocrit dominates
WHITE CELL INCLUSIONS, name 3
Erlichiosis

Histoplasmosis

Dohle bodies – sign of infection
The key clinical problem in AML
infection due to neutropenia
For AML, remission means that:
the tumor burden has gone from 1 trillion cells to 1 million cells.
The Ph1 chromosome is a __ translocation which creates a ___ oncogene.
9:22

bcr-abl
leukocyte alkaline phosphatase in CML
low
gleevec, what's it do, how's it work
The bcr-abl in CML codes for a tyrosine kinase, and gleevec inhibits the tyrosine kinase,
EVALUATION OF THE PATIENT WITH AN ELEVATE GRANULOCYTE COUNT, 5 steps
1) markedly elevated > 30,000? slightly elevated suggests infection

2) Are 5% of the cells myelocytes or younger? If so, consider CML even if the WBC is not markedly elevated

3) Examine the patient for splenomegaly (CML, Myelofibrosis)

4) If the diagnosis is not established at this point, do a marrow aspirate and biopsy, and obtain cytogenetics.

5) If it looks like CML but the Philadelphia chromosome is not seen, obtain tests for bcr-abl.
Neutropenia: Underproduction
7 conditions
1) Myelophthisic – leukemia, lymphoma, non-hematologic tumor (rare)

2) Myelodysplasia – a late manifestation of disease

3) Aplastic anemia (which is actually a pancytopenia)

4) Folate and B-12 deficiency (usually only an anemia, can be pancytopenia)

5) Cyclic neutropenia – rare

6) Congenital neutropenia

7) Chediak-Higashi syndrome (problem of WBC function/also low neutrophils)
Neutropenias of decreased survival, 4 conditions
1) Hypersplenism

2) Autoimmune neutropenia

3) Felty’s syndrome – (Rheumatoid arthritis, splenomegaly, neutropenia)

4) Large granular lymphocyte syndrome (a T/NK cell disorder often in association with rheumatoid arthritis)
Clinical problems of neutropenia, 3
1 Systemic infection

2 oral ulcers

3 ecthyma (due to pseudomonas)
Most common adult leukemia
CHRONIC LYMPHOCYTIC LEUKEMIA
staging of CLL
Stage 0
Elevated lymphocytes

Stage 1
As above plus adenopathy

Stage 2
As above plus hepatomegaly or splenomegaly

Stage 3
As above, plus anemia*

Stage 4
As above, plus thrombocytopenia*
Lab tests for EBV
Heterophile (mono spot) antibody to sheep red cells after absorption with guinea pig sera

Specific EBV tests:

EBV Capsid antigen: IgM at presentation lasts 4-6 weeks; IgG is lifelong

EBV Nuclear Antigen appears in 3-4 weeks, lifelong.
INFECTIOUS MONONUCLEOSIS
A self limited malignancy EBV infects lymphocytes.
2 major findings--physical and lab--usually found in hairy cell leukemia (besides morphology)
splenomegaly (can be massive) and pancytopenia
Lab tests for confirming Hairy cell leukemia, what antigens?
diagnosis confirmed by TRAP, or by flow cytometry
CD5 negative
CD 19 +
CD 20 +
CD 11b +
Therapy of hairy cell leukemia
2 CDA or 2-DCF
which immunoglobulins produced in multiple myeloma, by percentage
Approximately 60% are IgG kappa or lambda

Approximately 20% are IgA kappa or lambda

Approximately 20% are light chain only, i.e. kappa or lambda
(These patients have no serum monoclonal spike and have only a urine spike)
IgD kappa or lambda, IgE kappa or lambda, and non-secretory myeloma are RARE.
Clinical problems of multiple myeloma involve which three organ systems:
Lytic bone lesions: pain or fractures

Decreased normal globulins (and therefore bacterial infections)

Renal disease due to light chains plugging up renal tubules.
Lab features of multiple myeloma
1Normochromic normocytic anemia

Anemia of chronic disease (renal disease and malignancy)

Myelophthisic anemia

2) 80% of cases show a monoclonal spike on serum protein electrophoresis
Marrow of multiple myeloma
Marrow: Sheets of plasma cells, at least one-half of the marrow
WALDENSTROM’S MACROGLOBULINEMIA
Clonal disorder of plasmacytoid lymphocytes associated with IgM production.
major clinical manifestation of waldenstrom's macroglobulinemia
hyperviscosity
symptoms of hyperviscosity in waldenstrom's macroglobulinemia, 5
Hyperviscosity:
1 Weakness
2 fatigue
3 blurred vision
4 peripheral neuropathy
5 tinnitus
how high of an IgM in waldenstrom's macroglobulinemia will produce symptoms of hyperviscosity
IgM of 3000
blood smear of waldenstrom's macroglobulinemia
Normochromic normocytic smear. Rouleaux formation if IgM is extremely high.
Marrow in waldenstrom's macroglobulinemia
Marrow shows increase in plasmacytoid lymphs
MONOCLONAL GAMMOPATHY OF UNKNOWN SIGNIFICNACE

(previously called benign monoclonal gammopathy)
A monoclonal serum spike, or rarely monoclonal urine light chains, without proof of myeloma, i.e. the bone marrow contains elevated plasma cells but not enough for a diagnosis and there are no lytic lesions.
When trying to decide between myeloma and MGUS, 3 steps (pre-marrow)
1 An IgG > 3 (k?) or an IgA >2 suggests myeloma.

2 Decreased levels of the other immunoglobulins, i.e. decreased IgA and IgM in cases of elevated IgG, suggest myeloma. In MGUS the “other” globulins are usually normal.

3 Lytic bone lesions, unless due to another process, would establish myeloma.
2 most common B-Cell disorders resulting in impaired host defense
Chronic lymphocytic leukemia and myeloma
Diagnosis of B-cell disorders:
Quantitative IgG, IgA, IgM
Treatment of B cell disorders
Treatment of CLL or myeloma rarely corrects the defect

Immunoglobulin replacement IVIG 200 mg/kg q 4 weeks

Problem with replacing IgA deficient patients – allergic reactions

Treat the infections
Early complement deficiency: what infections
pyogenic infections
Late complement deficiency: what infections
neisseria infections
name the opportunistic parasites, 3
Pneumocystis carinii
toxoplasma gondi
cryptosporidium
opportunistic mycobacteria, 2
M tuberculosis
M. avium
opportunistic viruses, 6
1 Cytomegalovirus
2 herpes simplex
3 herpes zoster
4 Epstein-Barr virus
5Papovavirus-JC
6 adenvirus
opportunistic fungal infections, 4
1 Cryptococcus neoformans
2 histoplasmosis
3 candida, sp,
4 aspergillosis
opportunistic bacterial infections, 3
Nocardia, legionella, listeria
Diagnosis of T cell disorders
Quantitive T and B-lymphocytes

Skin test to recall antigens (mumps, TB)