WBC Disorders Flashcards
Common markers for Myeloblasts
1) MPO
2) CD45+
Cell marker for NK Cells
1) CD56
2) CD16 (Fc receptor)
Immature Lymphoblast will stain positive for what cell marker?
TdT+ (DNA Polymerase)
Describe ANC
Amount of Neutrophils and Bands * WBC count
ANC <500 is sus. to
Opportunistic Infections
General Overall causes of Neutropenia
1) Decreased Production
2) Increased Destruction
Causes of Lymphopenia
1) Autoimmune
2) immunodeficiency
3) Radiation/Chemo
4) Increased Corticosteroids
Mechanisms of Leukocytosis
1) Increased marrow production
2) increased release from marrow stores
3) Decreased Margination
4) Decreased Extravasation
Causes of Increased marrow production leading to leukocytosis
1) Chronic Inflammation –> Growth Factor Dependent
2) Paraneoplastic: Hodgkin Lymphoma –> Growth Factor Dependent
3) Myeloproliferative Neoplasms: Chronic Myeloid Leukemia (Growth Factor INDEPENDENT)
Cause of decrease margination leading to leukocytosis
Exercise and Catecholamines
Most common cause of cancer in children
Acute Lymphoblastic Leukemia/Lymphoma
Characteristics of Presentation of ALL
1) Fever - Decreased Neutrophils
2) Bleeding - Thrombocytopenia
3) Fatigue - Anemia
4) Pain - Marrow Hypercellularity
5) Dry Tap
List the Acute Leukemias
1) Acute Lymphoblastic Leukemia/Lymphoma
2) Acute Myeloid Leukemia
General Morphology of cells in Acute Leukemias
Large Immature Cells with poked out nuclei
Pathogenesis of Acute Leukemias
1) Gene Mutation
- Self-renewed capacity
- Maturation arrest
What is t(12:21)
Acute B-cell Leukemia/Lymphoma
-Good prognosis
What determines a bad prognosis in Acute Lymphoblastic Leukemia
1) Less than age 2
2) Adolescence/Adulthood
3) High WBC (>100K)
Hallmark Feature of Small Lymphocytic Lymphoma/Chronic Lymphocytic Leukemia on a blood smear
1) Small mature lymphocytes w/ Smudge Cells
Pathogenesis of CLL
1) De Novo Mutations
- Can lead to Diffuse Large B Cell Lymphoma via Richter Transformation
What mutations cause CLL to transform into DLBCL also known as a Richter Transformation
1) MYC
2) TP53
When thinking about the diagnosis of CLL what would you use?
What would you be looking for with what restriction?
1) Flow Cytometry
2) CD5+, CD19, and KAPPA Restricted
Patient Presentation of Follicular B Cell lymphoma
1) Generalized Painless Lymphadenopathy
A Patient has been diagnosed with Follicular B Cell Lymphoma a year ago. They come back into clinic for a check up and you notice that their generalized lymph adenopathy under the chin has gotten larger in size but is still painless. You are nervous the cells have undergone what transformation.
Richter Transformation into Diffuse Large B cell lymphoma
Translocation for Follicular Lymphoma
t(14:18) = IgH/BCL-2
Presentation of Chronic Lymphoblastic Leukemia/Small Lymphocytic Lymphoma
1) Increased mature lymphocytes (increased WBC)
- CD5+ and CD20+
2) Generalized Lymphadenopathy
Complications associated with Chronic Lymphoblastic Leukemia/Small Lymphocytic Lymphoma
1) Hypogammaglobinemia
2) Autoimmune Hemolytic Anemia
List the different links with Diffuse Large B Cell Lymphoma and list the lymphoma they cause
1) EBV: Immunodeficiency-related LCL
2) HHV-8: Primary Effusion Lymphoma
What somatic mutation presents in Diffuse Large B Cell Lymphoma
BCL-6 –> overexpression
Diagnosis of Diffuse Large B Cell Lymphoma
1) Enlarging LN or Spleen
Translocation of Burkitt Lymphoma
t(8:14) IgH/MYC
Types of Burkitt Lymphoma
1) African - Jaw (Children) - EBV
2) Sporadic - Mediastinal
3) HIV- related
Size of the cells in Burkitt Lymphoma
1) Intermediate
What is a helpful marker but not a definitive diagnosis for Burkitt Lymphoma
Ki-67 marker - associated w/ rapid cell proliferation
Most common malignancy between 15-19 is…
Hodgkin Lymphoma
Morphology of cells in Anaplastic Large-Cell Lymphoma
1) Donut Cells or Horseshoe cells
Pathogenesis of Marginal Cell Lymphoma
1) H. Pylori - Gastric MALToma
2) Hashimoto Thyroiditis - MZL of Thyroid
3) Chronic Sialadenitis - Salivary Gland MZL
Hair Cell Leukemia is common in ____
Adult Males
Presentation of Hair Cell Leukemia
1) Massive Splenomegaly
2) Dry Tap on Bone
Why does Hairy Cell Leukemia present with Massive Splenomegaly
1) Accumulation of Hairy Cells in Red Pulp
2) Marrow Fibrosis
Describe the morphology of the cells in Hairy Cell Leukemia
1) B Cells w/ Fried egg appearance
2) Tartrate-Resistant Acid Phosphatase (TRAP)
What is Hairy Cell Leukemia + for?
Tartrate-Resistant Acid Phosphatase
ALK tyrosine kinase constitutive active due to
Gene rearrangement
Presentation of Adult T Cell Leukemia
1) Skin Involvement: Exfoliation and popular lesions/ necrotizing nodules
2) Lytic Bone Lesions and Hypercalcemia
Morphology of Cells for Adult T Cell Leukemia
Cloverleaf Cells
What Leukemia is associated with HTLV-1
Adult T Cell Leukemia
Extranodal NK/T cell Lymphoma virus association
EBV
Clinical Presentation for Multiple Myeloma
1) Hypercalcemia
2) Renal Failure/Dysfunction
3) Anemia
4) Bone Lesions/Back Pain
What are Bence Jones Proteins
Protein in Urine via Light Chains in Multiple Myeloma
SPEP for Multiple Myeloma will present with
Either IgG or IgA
List the Classical Hodgkin Lymphomas
1) Nodular Sclerosis
2) Mixed Cellularity
3) Lymphocyte Rich
4) Lymphocyte Depleted
Hallmark cell of Hodgkin Cell Lymphoma
Reed Sternberg
Reed Sternberg Cells will stain positive for ….
1) CD15
2) CD30
3) Pax-5
B Symptoms are associated with what cancer
Hodgkin Lymphoma
What are the B Symptoms
1) Fever
2) Night Sweats
3) Unexplained Weight Loss
How do you diagnose Hodgkin Lymphoma
Staining
What type of cells are present in Nodular Lymphocyte Predominant Lymphoma
Popcorn Cells
Cell markers in Nodular Lymphocyte Predominant Lymphoma that is not characteristic of other Hodgkin Lymphomas
ONLY CD20+
- NOT CD30 and CD15
Patients with AML will present with …
Pancytopenia:
1) Fever
2) Fatigue
3) Bleeding/Bruising
What is t(8:21)
Translocation that causes AML in young patients
-Good prognosis
What is t(15:17)
Acute Promyelocytic Leukemia
___ will be present in cells in pts who have Acute Promyelocytic Leukemia
1) t(15:17)
2) Auer Rods
Fusion of what genes are present in AML that is caused by t(8:21)
RUNx1/Runx1T1
Patients who have multiple myeloma are at risk for a Thromboembolic Disorders because ….
Hyperviscosity
How do you treat Acute Promyelocytic Leukemia?
All Trans RA
Myeloproliferative Neoplasms are associated with mutated ____
Kinases
Describe the HCT of a pt that has CML
Increased Buffy Coat
List the Clnical Manifestations of CML
Splenomegaly - extramedullary hematopoiesis
Marked Leukocytosis
List the Myeloproliferative Neoplasms
1) Polycythemia Vera
2) Essential Thrombocythemia
3) Primary Myelofibrosis
4) CML
_____ MPS presents with massive splenomegaly due to depletion of marrow elements. You treat with Ruxolitinib
Myelofibrosis
What do you use to treat primary myelofibrosis
Ruxolitinib
Polycythemia Vera, Essential Thrombocythemia, and Primary Myelofibrosis all have a mutated
JAK2 Kinase
Chromosome Translocations of Philadelphia Chromosome
t(9:22); BCR-ABL
List the Langerhan Cell Histiocytosis cell morphology
1) Grooved nuclei of Langerhans Cells
2) Berback Granules
3) Stains S100 and CD1
List the Langerhans Cell Histiocytosis
1) Multifocal Multisystem Langerhans Cell Histiocytosis (Letterer-Siwe Disease)
2) Unisystem Langerhans Cell Histiocytosis (Eosinophilic Granuloma)
3) Pulmonary Langerhans Cell Histiocytosis