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MOHD III > Myeloid & Lymphoid disorders > Flashcards

Myeloid & Lymphoid disorders Flashcards

1
Q

Diffuse Large B-cell lymphoma (DLBCL)

A
  • Most common lymphoma in Western world*
  • diffuse proliferation of large neoplastic cells
  • 2x size as mature lymphocyte
  • ages: all (median late 60s)
  • possible translocations: MYC, BCL-2, BCL-6
  • express B-cell markers: CD19, CD20, CD22, CD79a, Pax-5
  • “B symptoms” common
  • prognosis: use anti-CD20 antibodies and multi-agent chemotherapy
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2
Q

Prognostic markers for DLBCL

A
  • international prognostic index IPI
  • cell of origin
  • double/triple hit lymphoma
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3
Q

most common lymphoma in children

A
  • hodgkin lymphoma

- Burkitt lymphoma

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4
Q

Acute Promyelocytic Leukemia

A
  • Subset of AML
  • myeloid cells proliferate but stop maturing when the cell is in the promyelocyte stage
  • Translocations: t(15;17); PML-RARA
  • responds to therapy with all-trans retinoic acid.
  • Treatment: ATRA (disassociates retinoic acid receptor complex and allows cell to continue differentiating)
  • PML-RARA activates tissue factor promotor leading to coagulation and increased risk of DIC (dissemenated intravascular coagulation)
  • expresses high levels of annexin II activating plasmin (leading to fibrinolysis)
  • “butterfly or dumbbell shaped” blasts (bilobed)
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5
Q

Follicular Lymphoma

A

most common indolent lymphoma (causing patients to present with widespread disease and high stage (III or IV)

  • affects predominantly adults (rarely kids)
  • densely packed lymphoid follicles (with minimal or no tingible body macrophages) containing small cleaved cells without nucleoli (centrocytes) and larger noncleaved cells with round to oval nuclei and multiple nucleoli (centroblasts)
  • positive B cell markers: CD19, CD20, bcl-6, CD10, Bcl-2
  • Translocation: t(14;18) causing rearrangement of BCL2 gene leading to over expression of Bcl-2 protein preventing apoptosis
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6
Q

Burkitt Lymphoma

A

aggressive (high-grade) B-cell lymphoma that is most common in children

  • translocation: t(8;14) in 75% of cases
    • t(2;8) t(8;22)
  • common in AIDS patients (CNS involvement common)
  • common where malaria is endemic
    • endemic BL = Ebstein-Bar virus present (95%)
    • endemic BL = presents with jaw and orbital bone tumors (60%)
  • Sporadic BL = presents with abdominal mass
  • “Starry Sky” morphology: tingible bodies (histiocytes) in sky of dark neoplastic lymphocytes; cells uniform in size and shape w/ 4-5 nucleoli
  • positive B-cell markers: CD19, CD20, CD22, CD79a, CD10, Bcl-6
    • NEGATIVE: Bcl-2 and TdT
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7
Q

Ebstein Bar Virus

A

infects lymphoid cells (B cells) by binding to the receptor for complement C3d (CD21)

  • causes Infectious mononucleosis
  • can cause Burkitt Lymphoma
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8
Q

Mantle Cell Lymphoma

A

low-grade non-Hodgkin lymphoma of B-cell neoplasm composed of monomorphous small-medium cells w/ irregular nuclei
- median age of 65–70 years
- mainly affects men
- patients present with advanced disease (stage III or IV)
Symptoms: lymphadenopathy, hepatosplenomagaly, bone marrow involvement, lymphocytosis, intestinal polyposis of any part of the GI tract
- proliferation may be in diffuse, nodular, or expansion of mantle zone
- scattered histiocytes with pink cytoplasm = common
Morphologic variants: Blastoid; Pleomorphic; small round lymphocytes; monocytoid differentiation
Positive B-cell markers: CD5, CD43, CD19, CD20, CD22, CD79a
NEGATIVE: CD23, CD10
Translocation: t(11;14)
- Positive for Cyclin D1 which induces cell growth

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9
Q

Marginal Zone B-cell Lymphomas

A

low-grade, B-cell, non-Hodgkin lymphoma that arises from the marginal zone of extranodal lymphoid follicles
3 types:
- mucosa-associated lymphoid tissue (MALT)
- nodal
- splenic
MALT lymphoma is the most common (usually in the stomach or small intestine) w/ median age: 61
- usually a history of chronic inflammation and autoimmune states: Helicobacter Pylori infection, Sjorgen syndrome, Hashimoto throiditis
- indolent clinical course w/ tendency to stay localized
- patients present usually at Stage I or II
Positive B-cell markers: CD19, CD20, CD22, CD79a
NEGATIVE: CD5, CD10, CD23, Blc-6, Cyclin D1

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10
Q

Classical Hodgkin Lymphoma

A
  • arises in lymph nodes (common in cervical region)
  • neoplastic tissues contain Hodgkin and Reed-Sternberg cells (HRS) residing in an infiltrate of inflammatory cells
  • bimodal age curve: peak at 15-35 years and at 60 years
  • higher incidence with history of infectious mononucleosis
  • patients present: enlarged lymph nodes; mediastinal mass (60%); B symptoms (40%)
  • associated with EBV infection and HIV infection
    Morphology: lymph nodes have “fish flesh appearance”
    Positive B-cell markers: CD30, CD15 (85%)
  • CD20 and CD79a positive in a third of patients
    NEGATIVE: CD45
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11
Q

Reed-Sternberg cells’ morphology in cHL

A

Large and binucleate with prominent eosnophilic nucleolus

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12
Q

Hodgkin cells’ morphology in cHL

A

Mononuclear or multilobated large cells

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13
Q

B-Cell Chronic Lymphocytic Leukemia

A

low-grade, non-Hodgkin, B-cell lymphoma

  • lymphoproliferative neoplasm of small mature appearing lymphocytes
  • affects mainly adults (median age: 65)
  • male predominance
  • most common chronic leukemia in western world
  • patient presents: fatigue, fever, night sweats, weight loss, autoimmune hemolytic anemia, decreased platelets, lymphadenopathy, infections, hepatosplenomegaly
  • blood smear shows lymphosytosis
    • lymphocytes are small with clumped chromatin and little cytoplasm
    • lymph nodes show pale proliferation centers
  • positive B-cell markers: CD19, CD20, CD22, CD79a
    • co-express CD5 and CD23
  • NEGATIVE: CD10 and Cyclin D1*
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14
Q

B-cell markers

A

CD19; CD22; CD20; CD21; CD79a; CD38; CD138; CD10; CD23

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15
Q

T cell lineage and NK cell markers

A

CD1alpha; CD2; CD3; CD5; CD7; CD4; CD8; CD16; CD56; CD57

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16
Q

immature lymphoid tissue marker

A

TdT (terminal deoxynucleotidyltransferase)
HLA-DR
CD34

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17
Q

Germinal center lymphocytes

A

are of B-lineage

  • express: CD19, CD20
  • co-express: bcl-6 and CD10
  • Negative for: bcl-2*
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18
Q

Reactive Lymphoid Hyperplasia (RLH)

A

enlargement of lymph nodes/lymphoid organs due to extensive proliferation of cellular compartments

  • unknown etiology
  • common in children
  • morphologic patterns seen in RLH include: follicular, sinusoidal, diffuse, and mixed
  • positive B-cell markers: CD19, CD20
    • co-express: bcl-6 and CD10
  • NEGATIVE: bcl-2*
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19
Q

Infectious Mononucleosis Lymphadenitis (aka Mono)

A

highly contagious acute condition caused by the Epstein-Barr virus (EBV)

  • affects primarily adolescents and young adults
  • peripheral blood: lymphocytosis w/ large, atypical B and T cells
  • after acute infection, converts to low grade, latent B-cell infection for life
  • symptoms: fever, pharyngitis, cervical/generalized lymphadenopathy
  • immunoblasts are a mixture of B (CD20, CD79a) and T cells (CD3)
  • hodgkin-like cells are negative for CD15 and CD30 but positive for B-cell markers
20
Q

Cat-Scratch Lymphadenitis

A

necrotizing granulomatous lymphadenitis caused by Bartonella henselae, a gram negative bacillus

  • 55% patients 18 years or younger
  • history of contact with cats
  • lymphadenopathy is unilateral (common: cervical, axillary, or inguinal lymph nodes)
  • lymph nodes are enlarged, matted, and adherent to surrounding tissue
  • symptoms: fever, malaise, headache, ache of bones and joints
  • has necrotizing granulomas: neutrophils become fragmented and fibrin is deposited with macrophages surrounding it forming a stellate shape
  • stain with Warthin-Starry silver stain
21
Q

Toxoplasma Lymphadenitis

A

caused by infection with protozoan Toxoplasma gondii
- most common parasitic infection in US
- lymphadenopathy common in posterior cervical region (may be tender or painless)
- symptoms: fever and myalgia
Characteristic Morphology:
1. follicular hyperplasia w/ larger germinal centers
2. numerous epithelioid histiocytes
3. aggregates of monocytoid cells

22
Q

Aplastic Anemia

A

anemia caused by bone marrow failure

  • “empty marrow”
    • all fat and no cells in bone marrow
23
Q

Granulocytic aplasia

A

lack of granulocytic precursors in marrow

24
Q

B12 deficiency

A

can lead to ineffective hematopoiesis
– defective DNA synthesis
– cytoplasm matures but nucleus remains immature
Megaloblastoid change = presence of abnormally large nucleated erythroblasts in the bone marrow

25
Q

Neutropenia

A

reduction in neutrophils in peripheral blood caused by:
- inadequate and ineffective granulopoiesis
- accelerated removal of neutrophils (by either infection or immune mediated)
- replacement of marrow by tumor
Patient presents: infections, ulcerating necrotic lesions on mouth and gums, necrotizing infections of lungs or urinary tract

26
Q

Neutrophilia

A

increase in number of circulating neutrophils

  • demargination (waiting neutrophils released from endothelium)
  • mobilization of maturation-storage compartment
  • increased production
  • myeloproliferatvie neoplasms
  • left shift
  • Toxic changes: seen with acute infections; includes: toxic granulation (coarse purple granulation), cytoplasm vacuolization (frothy cytoplasm), Dohle bodies (pale blue structures in cytoplasm)
27
Q

Leukemoid reaction

A
  • extreme leukocytosis
  • physiologic response to stress or infection
  • may resemble leukemia
28
Q

Acute Myeloid Leukemia

A

malignant neoplastic disease that arises from myeloid cell lines.

    • Characterized by the proliferation of myeloblasts in the bone marrow that are subsequently released into the bloodstream.
    • Excessive proliferation of immature blasts in the bone marrow impairs all other cell lines, resulting in anemia, clotting disorders, and increased susceptibility to infections.
  • Most common adult leukemia
  • expresses positive myeloid antigens on flow cytometry: CD13, CD33, CD117
  • may have Auer Rods: needle like accumulation of red granular material (indicative of myeloid blasts)
  • positive for Myeloperoxidase stain (indicative of myeloid blasts)
  • symptoms: fatigue (anemia), infections (neutropenia), bleeding (thrombocytopenia)
  • peripheral blood: leukocytosis
  • occassionally presents with pancytopenia
29
Q

AML with t(16;16)

A

abnormal immature eosinophils with dual granules (pink and purple granules in cytoplasm)

  • monocytic cells with myeloid blasts
  • infiltration of gums, skin, CNS more common than other AMLs
  • younger patients usually with good prognosis, but may relapse with myeloid sarcomas or CNS involvement
30
Q

Acute Megakaryoblastic Leukemia

A

Leukemia with megakaryoblasts that have cytoplasmic blebs which break off to become hypogranular platelets

31
Q

Acute Lymphoblastic Leukemia

A

malignant neoplasm of lymphoid stem cells

  • most common cancer in children ages 1-7
  • accumulation of lymphoblasts with loss of normal hematopoietic cells
  • peripheral blood: may show increased blasts or rare blasts
  • anemia and thrombocytopenia
  • symptoms: fatigue, infections, bleeding, Bone Pain (due to expansion of marrow putting pressure on nerves), hepatosplenomegaly, CNS symptoms (due to infiltration of cerebral spinal fluid)
  • flow cytometry used to determine lineage (B or T cell)
32
Q

B-ALL

A

B cell lineage of Acute lymphoblastic leukemia

  • peak incidence around age 3
  • loss of function mutations in genes required fro B cell differentiation (PAX5)
  • Trisomies of chromosomes 4 and 10 common in children with favorable prognosis
33
Q

T-ALL

A

T cell lineage of Acute lymphoblastic leukemia

  • common presentation: mediastinal mass in adolescent . male
  • 70% have gain of function mutation of NOTCH1, gene that’s essential for T cell differentiation
34
Q

Chronic Myeloid Leukemia

A

Chronic myeloproliferative disorder with increased neutrophils and granulocytic precursors
- basophilia always present
- median age: 55
- often asymptomatic but may present: lethargy, fatigue, weight loss
- splenomegaly
- peripheral blood: leukocytosis with left shift and basophilia
- hypercellular bone marrow w/ hypolobate megakaryocytes and increased myeloid:erythoid ratio
Translocation: t(9;22) causing a shortened Philadelphia chromosome 22 that results in the formation of a BCR-ABL fusion gene which is constitutively activated leading to proliferaton and differentiation
- Treatment: imatinib (Gleevec) (binds to ATP binding domain of BCR-ABL and prevents phosphorylation)

35
Q

Chronic Lymphocytic Leukemia (CLL)

A

Clonal lymphoproliferative disorder of mature lymphocytes

  • indolent leukemia of patients >60 years old
  • more common in males
  • B-cell neoplasms
    • positive for B-cell markers: CD19, CD20, CD22
  • Express CD5 (even though more typical of T cells)
  • complete expression of either monotypic kappa or lambda light chains indicates clonal expansion bc normal cells express a mixture of both light chains
  • peripheral blood: Lymphocytosis w/ clumped chromatin and smudge cells (degenerating nuclear material of dying lymphocytes)
  • generalized lymphadenopathy (50-60%)
  • increased susceptibility to infections due to hypogammaglobulinemia (insufficient numbers of B cells)
  • may develop immune mediated anemia and/or thrombocytopenia
  • expresses high levels of BCL2 (inhibits apoptosis) and BCR (signaling through Bruton tyrosine kinase) promoting survival of CLL/SLL cells
36
Q

Myelodysplastic Syndromes (MDS)

A

Clonal myeloid stem cell disorders characterized by cytopenias, ineffective hematopoiesis (hypercellular marrow), and dyspoiesis (ring sideroblasts in RBCs)

  • occurs in elderly (50-70yrs)
  • 50% present w/ infections, hemorrhage, fatigue
  • 50% asymptomatic
  • peripheral blood: pancytopenia w/ dyspoietic features of RBCs, granulocytes, platelets/megakaryocytes
  • may have increased myeloblasts (but <20% more = AML)
  • Dimorphic RBCs (mix of normochromic/cytic and macrocytic and hypochromic microcytic)
  • Trisomies and deletions are common (chromosome 5 and 7)
  • survival: 9-29 months
37
Q

Chronic Myeloproliferative Neoplasms (MPN)

A

clonal proliferations of multipotent myeloid stem cells resulting in increased number of differentiated granulocytes, RBCs, or platelets

38
Q

Essential Thrombocythemia

A

chronic MPN (myeloproliferative neoplasm)

  • thrombocytosis
  • increased megakaryocytes in bone marrow
  • causes thrombosis or hemorrhage (bc platelets not functioning correctly)
  • splenomegaly (50%)
  • occurs in adults (50-60 yrs)
  • indolent disorder with good prognosis
  • rarely transforms to AML or myelofibrosis
  • peripheral blood: large atypical megakaryocyte with lots of branches in the nuclear shape
39
Q

Primary myelofibrosis

A

clonal MPN with proliferation of predominantly megakaryocytes and granulocytes in marrow
- fully developed disease = reactive deposition of fibrous connective tissue and extramedullary hematopoiesis (resulting in Splenomegaly)
- occurs in middle age and elderly
- median survival = 3 years
- anemia with teardrop RBCs (due to marrow fibrosis)
- leukoerythroblastic reaction = immature myeloid cells and nucleated RBCs circulating in peripheral blood
Bone marrow:
– cellular stage: hypercellular w/ increased neutrophilsand clusters of large atypical megakaryocytes
– fibrotic stage: hypocellular

40
Q

JAK2 V617F

A

mutation in JAK2 gene

  • leads to increased proliferation of bone marrow
  • seen in most patients with: Polycythemia Vera
  • seen in 50% of patients with: Essential Thrombocythemia or Primary Myelofibrosis
41
Q

Plasma cell neoplasms

A

clonal proliferations of terminally differentiated B cells .
- secretes a monoclonal immunoglobulin called M-protein or monoclonal protein (tested by serum electrophoresis)

42
Q

Monoclonal Gammopathy of Undetermined Significance

A

type of plasma cell neoplasm

  • clinically asymptomatic
  • small increases in monoclonal protein
  • usually identified incidentally
  • 25% progress to plasma cell myeloma over 20 years
43
Q

Solitary Plasmacytoma

A

type of plasma cell neoplasm

  • growth of plasma cells either outside of bone marrow, extraosseous (lung, pharynx, or nasal sinuses) or a localized growth in marrow
    • no generalized involvement of marrow (thus have to biopsy the actual lesion)
  • treatment: local radiation
44
Q

Plasma Cell Myeloma

A

type of plasma cell neoplasm
- originates in bone marrow
- monoclonal serum that complete expression of either IgG, IgA
- protein (light chains) in urine
Symptoms: End organ damage, CARB (hypercalcemia, renal insufficiency, anemia, bony lytic lesions (softened bone appearing as darkish holes on XRay)), recurrent infections
Asymptomatic (“Smoldering”): M-protein in serum (>30g/dL) and bone marrow involvement (>10%) –> MGUS will have less
- peripheral blood: Rouleaux (linear array of RBCs indicative of increased plasma proteins)
Treatment: minimize end organ effect but not curative

45
Q

Primary Amyloidosis

A

type of plasma cell neoplasm

  • secretes intact or fragments of abnormal immunoglobulin light chains, amyloid, which deposits in tissues
  • causes end organ failure
  • deposits in: subcutaneous fat, kidney, heart, liver, GI, nerves, marrow, vessels

MOHD III (5 decks)

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