-
What disorder originates in the bone marrow and is initially systemic?
Leukemia
-
What disorder originates in the lymphoid tissues and is initially localized?
Lymphoma
-
Name some of the theories behind oncogenic activation
- Viral - viruses can suppress immune function or activate oncogenes (HTLV-I, II, V) and HIV-I
- Bone marrow damage
- Chromosome defects
- Genetic factors
- Immune dysfunction
-
What test is diagnostic for acute promyelocytic leukemia?
t(15;17)
-
How are malignant disorders classified and name some
- Classified by stem cell cell involved and length of clinical duration
- Lymphoproliferative disorders: actue or chronic
- Myeloproliferative disorders: acute or chronic
-
What specimens are used to aid in diagnosis is malignant disorders?
- Bone marrow exam - aspirate and biospy
- Peripheral blood exam - unexplained cytopenias
- Ongoing monitoring of response to therapy in patients with malignancy
- Assesses for cellularity, M:E ratio, megakaryocyte evaluation, iron stores, differential
- Flow cytomety, cytogenetics, molecular, and microbiology
-
What sites are used for bone marrow biopsies?
- Posterior superior iliac crest - most common
- Anterior iliac crest
- Sternum
-
How long is the duration for acute and chronic leukemias?
- Acute: weeks to months without treatment, death is due to infection and bleeding
- Chronic: Years without treatment
-
What is the predominant cell type in Acute and Chronic leukemias?
- Acute: Immature/blasts cells predominant
- AML: myeloblasts
- ALL: lymphoblasts
- Chronic: Maturing or mature cells predominate
- CML: granulocytes
- CLL: lymphocytes
-
What is the clinical and laboratory findings associated with acute leukemia?
- Sudden onset: affecting all ages
- Weakness and fatigue due to anemia
- petechiae and bruising due to thrombocytopenia
- Fever and infection due to neutropenia
- Variable leukocyte count:
- - WHO: Marrow blasts >/=20%
- - FAB - Marrow blasts >30%
- - with cellularity >70%
-
What is the clinical and laboratory findings associated with chronic leukemia?
- Frequently asymptomatic initially: affects adults
- Anemia: mild or absent
- Platelet: normal to slightly increased
- WBC: high
- Marrow cellularity is >70%
-
What type of treatment is used for acute and chronic leukemias?
- Chemotherapy used is dependent on type of leukemia - proper diagnosis is crucial
- Radiation
- Bone marrow/stem cell transplant
- Supportive with transfusions of RBCs and platelets, antibiotics, and growth factors
-
What is myeloperoxidase (MPO) stain used for?
- Auer rods stain positive
- Lymphocytic cells are negative for this stain
- Used to differentiate blasts of acute myelogenous leukemias (AMLs) from acute lymphoblastic leukemias (ALLs)
-
What is Sudan Black B stain used for?
- Stains phopholipids and lipoproteins
- Granulocytic cells and auer rods stain positive
- Lymphocytic cells are negative
- Used to differentiate blasts of AML from ALL
-
What is specific esterase stain (naphthol AS-D choloracetate esterase) used for?
- Detects esterase enzyme present in primary granules of granulocytic cells
- Monocytic cells negative for this stain
-
What is the nonspecific stain (alpha napthyl acetate and alpha napthyl butyrate) used for?
- Detects esterase enzyme present in monocytic cells
- Granulocytic cells are negative for this stain
-
What are the esterase stains useful in distinguishing?
Acute leukemias that are of myeloid origin from those leukemias that are of monocytic origin
-
What is Periodic acid-Schiff (PAS) stain used for?
- Stains intracellular glycogen bright pink
- Immature lymphoid cells, malignant erythroblasts, and megakaryocytic cells stain positive
- Myeloblasts and normal erythrocytic cells are negative
- Useful in diagnosis of erythroleukemia and acute lympoblastic leukemia
-
What is Leukocyte alkaline phosphatase (LAP) stain used for?
- Detects alkaline phosphatase enzyme activity in primary granules of neutrophils
- Use to differentiate chronic myelogenous leukemia (CML) from neutrophilic leukemoid reaction (NLR)
-
Describe the LAP (leukocyte alkaline phosphatase) score and the reference range
- 100 neutrophils are graded on a scale of 0-4+ based on stain intensity and size of granules
- Results are added together
- Reference range is 13-130
-
What is the clinical significance of a decreased LAP score?
- CML
- Paryoxysmal nocturnal hemoglobinuria
-
What is the clinical significance of an increased LAP score?
- Neutrophilic leukemoid reaction
- Polycythemia vera
- CML in blast crisis
- Late trimester pregnancy
-
What is the tartrate-resistant acid phophatase stain (TRAP) used for?
- Only hairy cells from hairy cell leukemia are resistant to inhibitiion with tartrate and continue to stain positive
- All other cells negative
-
What is Perl's Prussian blue stain used for?
Free iron precipitates, cells called siderocytes (pappenheimer bodies with Wright's stain)
-
What is associated with increased percentage of siderocytes?
- Severe hemolytic anemias
- Iron overload
- Sideroblastic anemia
- Post splenectomy
- Ringed sideroblasts seen in bone marrow of myelodysplastic syndrome
-
What condition:
Symptoms: Fever, Bone/joint pain, Bleeding, Hepatosplenomegaly
Neutropenia
Anemia
Thrombocytopenia
Variable WBC count
hypercellular marrow with bone marrow blasts >/=20%
Lymphoblasts stain PAS POS
Acute Lympoproliferative Disorders
-
What FAB classification has the following findings:
Most common childhood leukemia
Small lymphoblasts, homogeneous appearance
Best prognosis
Most T cell ALLs
FAB L1
-
What FAB classification has the following findings:
Most common in adults
Large lymphoblasts, heterogeneous appearance
FAB L2
-
What FAB classification has the following findings:
Leukemic phase of Burkitt lymphoma
Seen in both adults and children
Lymphoblasts are large and uniform with prominent nucleoli, cytoplasm stains deeply basophilic and may show vacuoles
Poor prognosis
B Cell lineage
FAB L3
-
What acute lymphoproliferative disorder has the following findings:
High-grade non-Hodgkin lymphoma phase of FAB L3 leukemia
Endemic in East Africa with high association with Epstein-Barr virus
Children present with jaw/facial bone tumors
US variant seen in children and young adults, present with abdominal mass
Burkitt lymphoma
-
What are the CD markers of progenitor B cells?
-
What are the most common subtype and early pre-B cells CD markers?
-
What are the CD markers of Pre-B cells and the 2nd most common subtype?
-
What are the CD markers of B cells?
-
What CD marker is present in all stages of B cells?
CD19
-
What genetic translocation is associated with FAB L3/Burkitt Lymphoma?
t(8;14) with rearrangement of the MYC oncogene
-
What genetic translocation is associated with Pre-B cell ALL?
t(9;22)
-
What genetic translocation is associated with B cell ALL?
t(4;11)
-
What genetic translocation is associated with T cell ALL?
t(7;11)
-
What condition:
Bone marrow: hypercellular
Blood: absolute lymphocytosis of >5, homogeneous, small, hyperclumped lymphocytes, and smudge cells
Chronic Lymphocytic Leukemia (CLL)
-
What condition:
B cell malignancy (CD19, CD20 POS)
Massive splenomegaly, extensive bone marrow involvement results in dry tap on bone marrow aspiration
Pancytopenia
Cytoplasm of lymphocytes shows hair-like projections
Tartrate resistant acid phosphatase (TRAP) stain positive
Hairy cell leukemia (HCL)
-
What condition:
B cell or T cell malignancy
Marked splenomegaly
Lymphocytosis >100 with many prolymphs
Anemia
Thrombocytopenia
Prolymphocytic leukemia (PLL)
-
Name the following cells identified with the following CD Markers:
CD2, CD3
CD4
CD8
CD13
CD11c, CD14
CD19, CD20
CD33
CD34
CD16, CD56
-
Name what the following cytochemical stains identify:
Myeloperoxidase
Sudan black B
Specific esterase stain
Non-Specific esterase stain
TdT
PAS (Periodic Acid-Schiff)
TRAP (Tartrate resistant acid phosphatase)
LAP
|
|