MDS, MPNs, Lymphomas, and Plasma Cell Malignancies Flashcards
Features of MDS (2)
Ineffective hematopoiesis, Increased risk of transformation to AML
Two scenarios of MDS
Idiopathic (primary), Therapy-related (secondary
Hypolobation of mature neutrophils (including bilobed nuclei: pseudo-Pelger-Huet cells), hypogranularity of cytoplasm
Dysgranulopoiesis
2 reasons for the frequent occurrence of splenomegaly and hepatomegaly in patients with MPNs
Sequestration of excess blood cells, extramedullary hematopoiesis
3 possible negative end points for MPNs
Transformation to acute leukemia, Transformation to MDS, Excessive marrow fibrosis (resultant marrow failure)
4 Causes of Myelodysplasia that may mimic MDS
drugs, deficiency of B12, folate, or other nutrients, viral infection, toxin exposure
BCL1 is characteristic of _____.
Mantle Cell Lymphoma
BCL2 is characteristic of ______.
Follicular Lymphoma
CHL: both sides of diaphragm, lack collagen bands, EBV+ (75%)
Mixed Cellularity CHL
CHL: found in children and older patients
Mixed Cellularity Hodgkin lymphoma
CHL: least common of 4 subtypes, usually EBV+, numerous RS cells that may appear anaplastic and bizarre
Lymphocyte Depleted CHL
CHL: most common subtype
Nodular Sclerosis HL
CHL: nodular growth pattern with residual germinal centers, RS cells present but rare
Lymphocyte Rich CHL
CHL: predominates in young adults, especially females
Nodular Sclerosis HL
CHL: thickened lymph node capsule with broad bands of collagen and nodular areas surrounded by broad collagen
Nodular Sclerosis HL
CLL/SLL is more common in ______ (sex).
males (2:1)
CLL/SLL is most common in ______ (demographic).
older adults (Median age: 65)
Cytogenetic findings in MDS (5)
Monosomy 7; Deletion 7q; Monosomy 5; Deletion 5q; Trisomy 8
Low Grade MDS: Anemia, increased platelets, and distinctive megakaryocytes with small, round, non-lobated nuclei
Isolated Deletion of 5q
Low Grade MDS: Good prognosis, median survival >5 years
Refractory Cytopenia with Unilineage Dysplasia
Low Grade MDS: Median survival 2.5 years, 10% transformation to AML in 2 years
Refractory Cytopenia with Multilineage Dysplasia
Low Grade MDS: myeloblasts account for less than __% of marrow cells and less than __% of peripheral blood cells
5; 2
MDS is most common in ______ (demographic)
older adults
Megakaryocytes with hypolobated or non-lobated nuclei, often hyperchromatic nuclei, often megakaryocytes with small size
Dysmegakaryopoiesis
Most common cause of death in polycythemia vera patients, including locations (3): _____
Thrombosis (Mesenteric vein, Splenic vein, Portal vein)
Most common leukemia in the western world
Chronic Lymphocytic Leukemia (CLL)
MPN Genetic Findings: BCR-ABL1 fusion gene on der22
Chronic Myelogenous Leukemia
MPN Genetic Findings: JAK2 mutations in 50% of cases
Polycythemia Vera, Primary Myelofibrosis, and Essential Thrombocytopenia
MPN Morphology: clusters of large bizarre megakaryocytes followed by reticulin fibrosis with loss of marrow space
Primary Myelofibrosis
MPN Morphology: Dacryocytes in peripheral blood
Primary Myelofibrosis
MPN Morphology: hypercellular marrow with small megakaryocytes containing round non-lobated nuclei
Chronic Myelogenous Leukemia
MPN Morphology: large bizarre megakaryocytes
Polycythemia Vera and Essential Thrombocytopenia
MPN Prognosis: 10-20 years or more
Polycythemia Vera and Essential Thrombocytopenia
MPN Prognosis: 2-3 years (untreated)
Chronic Myelogenous Leukemia
MPN Treatment: PTKIs
Chronic Myelogenous Leukemia
MPN Treatment: serial phlebotomy, aspiring therapy, mild chemotherapeutic drugs
Polycythemia Vera
MPN: characterized by trilineage hyperplasia with erythrocytosis
Polycythemia Vera
MPN: Neutrophilia, Basophilia, and Thrombocytosis
Chronic Myelogenous Leukemia and Primary Myelofibrosis
MPN: sustained marked thrombocytosis
Essential Thrombocytopenia
MYC is characteristic of ______.
Burkitt Lymphoma
NHL Cytogenetic Findigns t(11;14)(q13;q32)
Mantle Cell Lymphoma
NHL Cytogenetic Findings: Burkitt Lymphoma
t(8;14)(q24;q32), MYC gene
NHL Cytogenetic Findings: CLL/SLL
del13q14, Trisomy 12, del11q22-23, del17p13
NHL Cytogenetic Findings: CLL/SLL: least common and worst prognosis
del17p13
NHL Cytogenetic Findings: CLL/SLL: most common and best prognosis
del13q14
NHL Cytogenetic Findings: del13q14
CLL/SLL
NHL Cytogenetic Findings: Follicular Lymphoma
t(14;18)(q32;q21), BCL2 fusion gene
NHL Cytogenetic findings: Mantle Cell Lymphoma
t11;14)(q13;q32)
NHL Cytogenetic Findings: t(14;18)(q32;q21), BCL2 fusion gene
Follicular Lymphoma
NHL Cytogenetic Findings: t(8;14)(q24;q32), MYC gene
Burkitt Lymphoma
NHL Immunophenotype: CD19+, CD20+ only
Burkitt Lymphoma
NHL Immunophenotype: CD19+, CD20+, CD10+, BCL6+, BCL1,+, CD5+
Mantle Cell Lymphoma
NHL Immunophenotype: CD19+, CD20+, CD10+, BCL6+, BCL2+
Follicular Lymphoma
NHL Immunophenotype: CD5+, CD19+, CD23+, CD20+ (weak), sIgG (weak)
CLL/SLL
NHL Immunophenotype: Germinal Center markers
CD10+, BCL6+
NHL symptoms: Burkitt presents in jaw/facial bones, distal ileum cecum, and omentum (______) or iliocecal (______)
Endemic; Sporadic
NHL symptoms: highly aggressive with endemic and sporadic types
Burkitt Lymphoma
NHL symptoms: Lymphadenopathy with massive splenomegaly (50%) and peripheral blood involvement (25%)
Mantle Cell Lymphoma
NHL symptoms: most closely associated with EBV+, especially in malaria belt
Burkitt Lymphoma
NHL symptoms: mostly asymptomatic except for lymphadenopathy
CLL/SLL and Follicular Lymphoma
Non-Hodgkin lymphoma Morphology: germinal center B cell lymphoma, lymph node architectural effacement, closely packed follicles with scant mantle zone
Follicular Lymphoma
Non-Hodgkin lymphoma Morphology: scant, agranular cytoplasm; monoclonal mature lymphocytes, lymph node architectural effacement
CLL/SLL
Non-Hodgkin lymphoma Morphology: small to medium B lymphocytic neoplasm, effacement of lymph node architecture, starry sky appearance
Mantle Cell Lymphoma
Non-Hodgkin lymphoma Morphology: starry sky pattern, abundant basophilic cytoplasm, multiple basophilic medium-sized nucleoli
Burkitt Lymphoma
Non-Hodgkin lymphoma most likely to be seen in children
Burkitt Lymphoma
Plasma Cell Malignancy: 75% of lesions occur in upper respiratory tract
Extraosseus Plasmacytoma
Plasma Cell Malignancy: bone marrow-based, multifocal plasma cell neoplasm
Plasma Cell Myeloma (PCM)
Plasma Cell Malignancy: bone pain in back or extremities, weakness and fatigue
Plasma Cell Myeloma (PCM)
Plasma Cell Malignancy: high M protein in urine
Plasma Cell Myeloma (PCM)
Plasma Cell Malignancy: localized plasma cell tumors in tissues outside bone marrow
Extraosseus Plasmacytoma
Plasma Cell Malignancy: localized tumor of bone, consisting of clonal plasma cells
Solitary Plasmacytoma of Bone
Plasma Cell Malignancy: male predominance
Extraosseus Plasmacytoma
Plasma Cell Malignancy: Marrow plasmacytosis <10%, no lytic bone lesions
Monoclonal Gammopathy of Undetermined Significance (MGUS)
Plasma Cell Malignancy: Monoclonal antibody with no evidence of PCM or other causes
Monoclonal Gammopathy of Undetermined Significance (MGUS)
Plasma Cell Malignancy: most common gammopathy
Monoclonal Gammopathy of Undetermined Significance (MGUS)
Plasma Cell Malignancy: Rouleaux formation present in peripheral smear
Plasma Cell Myeloma (PCM)
Plasma Cell Malignancy: single bone lesion
Solitary Plasmacytoma of Bone
Plasma Cell Malignancy: Symptoms of PCM (4)
Hypercalcemia, Renal Insufficiency, Anemia, Bone lesions
Proliferation of one or more myeloid lineages with increases in one or more blood cell types
Myeloproliferative Neoplasm
RBC precursors with nuclear budding, irregularly-shaped nuclei, lack of coordination between nuclear and cytoplasmic maturation, increased ring sideroblasts
Dyserythropoiesis
Refractory Anemia with Excess Blasts-__ (RAEB-__): ____% blasts in marrow or ____% blasts in peripheral blood; median survival of 16 months
1; 1; 5-9; 2-5
Refractory Anemia with Excess Blasts-__ (RAEB-__): ____% blasts in marrow or ____% blasts in peripheral blood; median survival of 9 months
2; 2; 10-19; 5-19
RS Cells: Common Features (4)
Large (up to 100mcm), multiple nuclei or large loblulated nucleus, large eosinophilic nucleolus, ample cytoplasm
RS Cells: Important Antigens
CD15+, CD30+, CD45(-)
Types of High Grade MDS
Refractory Anemia with Excess Blasts -1 and -2 (RAEB-1 and RAEB-2)