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47 Cards in this Set
- Front
- Back
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Plasma Cell Myeloma
Clinical Features |
- Accounts for 15% of all hematologic malignancies
- Bone involvement - Bone fractures, pain - Appear radiographically as MULTIPLE PUNCHED-OUT, LYTIC BONE LESIONS - Monoclonal serum gammopathy - Bence Jones Proteins |
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Bence Jones proteins
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- found in urine
- are toxic to the renal tubular - Monoclonal immunoglobulin light chains |
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What are common sites of bone involvement in plasma cell myeloma?
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spine 66%
ribs 44% skull 41% pelvis 28% femur 24% clavicle 10% scapula 10% |
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Who gets Plasma Cell Myeloma?
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Adults (m=69y)
M>F B>W |
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What is myeloma diagnosis beased on?
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integrationg of clinical, radiological and pathological features
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Plasma Cell Myeloma
Pathology (Morphology, Phenotype, Genotype) |
- Morphology
- Marrow plasmacytosis >30% - Plasma cells with abundant cytoplasm, a perinuclear hof (prominent Golgi) and eccentric nuclei. - Phenotype - CD38 - CD138 - Negative for B-cell markers - Genotype - Ig clonally rearranged - Complex cytogenetic abnormalities; no specific changes |
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Plasma Cell Myeloma
Prognosis |
Usually aggressive incurable clinical course
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Histologic Findings in
Burkitt's Lymphoma |
Diffuse proliferation of small, noncleaved cells exhibiting “starry sky” pattern with abundant cellular debris within macrophages. Small-medium round cells with a thin rim of basophilic cytoplasm containing numerous vacuoles.
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Burkitt's Lymphoma
Etiology |
Specific reciprocal translocations involving C-MYC gene: 8q24
- Deregulation integral to lymphomagenesis - Encodes transcription factor involved in control of cell proliferation - EBV plays important but unknown role in pathogenesis |
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Burkitt's Lymphoma
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B-cell non-Hodgkin lymphoma (NHL)
- Represents only 1-2% of all lymphomas - Accounts for 30-50% of all childhood lymphoma - Male sex predilection (M:F 2.5) - Highly aggressive, often presenting at extranodal sites or as acute leukemia - Large, bulky masses, rapidly growing - Fastest growing malignancy in humans (short doubling time) - Advanced stage III/IV in 70% cases |
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African/Endemic Burkitt Lymphoma
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- Presents most often in head/neck, e.g. jaw/orbit
- Mean age in Africa is 7 years - EBV-associated 100% |
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Western/Sporadic Burkitt Lymphoma
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- Presents primarily as rapidly expanding abdominal mass, arising from ileocecal GI structures (intussusception common)
- Mean age of 11 years - EBV-associated 30% |
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Immunodeficiency associated Burkitt Lymphoma
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- Presents primarily as nodal disease +/- bone marrow involvement
- Adults, may be initial manifestation of AIDS - EBV-associated 25-40% |
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Translocations In Burkitt's Lymphoma
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- t(8;14)(q24;q32) c-myc/IgH [90% of cases]
- t(2;8)(q11;q24) c-myc/Igkappa [5%] - t(8;22)(q24;q11) c-myc Iglambda [5%] |
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Burkitt's Lymphoma
Surface Antigens |
CD19, CD20, Igkappa/lambda
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Burkitt's Lymphoma
Morphology |
- Diffuse, monomorphic cells, with cytoplasmic lipid vacuoles
- Numerous mitotic figures - “Starry sky” with apoptotic debris |
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Burkitt Lymphoma
Prognosis |
- Potentially curable with intensive chemotherapy
- Treatment begins ASAP due to rapid doubling time of tumor - Most patients also receive CNS prophylaxis with intrathecal methotrexate and craniospinal radiation - Current 3 year relapse free survival rate - 90% for patients with local disease - 60-80% in advanced stage, including Burkitt leukemia. |
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Hairy Cell Leukemia
Etiology |
- Undetermined - ?? exposures to benzene, insecticides, solvents
- Clonal B-cells infiltrate BM/spleen and interfere with function, resulting in BM failure and organomegaly. |
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Hairy Cell Leukemia
Presentation |
- Monocytopenia is characteristic
- Weakness/fatigue; easy bruising - Recurrent opportunistic infections, vasculitis, immune dysfunction |
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Hairy Cell Leukemia
Phenotype (antigens) |
- CD19, CD20, sIg
- Unique markers expressed: CD11c, CD25, CD103 - Strong positivity for tartrate-resistant acid phosphatase (TRAP) |
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Hairy Cell Leukemia
Peripheral Blood |
- circulating cells with characteristic cytoplasmic
projections, which appear as fine (hairlike) microvilli |
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Hairy Cell Leukemia
Bone marrow |
- “Dry tap” aspirate due to increased reticulin fibrosis
- Infiltration with a mononuclear cells with a fried-egg appearance |
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Hairy cell leukemia
clinical course |
- chronic/indolent
- Most patients achieve clinical remissions and long-term cures - Although relapses are known to occur after 5-10 years, they usually are responsive to the same treatment |
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Hairy cell leukemia
first-line therapy |
2-chlorodeoxyadenosine (2-CdA)
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Is splenectomy used in treatment of Hairy Cell Leukemia?
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- The first standard treatment modality used commonly IN THE PAST but has been replaced by the newer agents available
- CURRENTLY, reserved for patients whose conditions fail to respond to systemic therapy, or those with active infections and bleeding from thrombocytopenia. |
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Mycosic Fungoides
Course |
Present with cutaneous plaques/patches
Long natural history – years Slow dissemination |
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Sezary Syndrome
Course |
- GENERALIZED ORGAN INVOLVEMENT:
- Skin, peripheral blood, lymph nodes, visceral organs - Pruritis, alopecia, palmar hyperkeratosis, onychodystrophy - Aggressive course |
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Mycosis Fungoides/Sezary Syndrome
Leukemia Information |
- Rare, accounts for 0.5% of NHL
- Most common cutaneous T-cell NHL - Adults/elderly; M>F |
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Mycosic Fungoides
Skin |
- Dermal infiltration of medium-sized “cerebriform” cells
- Pautrier microabscess - Epidermotropism common |
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Mycosic Fungoides
Phenotype |
CD4+
CD7- (Helper T cells without CD7) |
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Reed-Sternberg cells
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- pale-staining acidophilic cytoplasm and one or two nuclei with peripheral chromatin clumping and unusually conspicuous acidophilic nucleoli.
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What are the WHO classifications of Hodgkin Lymphoma?
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1. Nodular lymphocyte-predominant Hodgkin lymphoma
2. Classical Hodgkin lymphoma (CHL) (95% of all HL) |
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Classical Hodgkin Lymphomas
Four Histological subtypes |
Immunophenotypic and genetic features of the neoplastic cells are identical in all four subtypes
- Nodular sclerosis classical Hodgkin lymphoma (NSHL) - Mixed cellularity classical Hodgkin lymphoma (MCHL) - Lymphocyte depleted classical Hodgkin lymphoma (LDCHL) - Lymphocyte rich classical Hodgkin lymphoma (LRHL) |
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Classical Hodgkin Lymphomas
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Neoplasm composed of mononuclear Hodgkin cells and multinucleated Reed-Sternberg (RS) cells residing in an infiltrate containing a variable mix of non-neoplastic small lymphocytes, eosinophils, neutrophils, histiocytes, plasma cells, fibroblasts and collagen fibers.
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Clinical Stage 1
of Hodgkin and Non Hodgkin Lymphoma |
Involvement of a single LN region (I) or involvement in a single extralymphatic organ or site (IE)
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Clinical Stage 2
of Hodgkin and Non Hodgkin Lymphoma |
Involvement of 2 or more LN regions on the same side of the diaphragm alone (II) or with involvement of limited contiguous extralymphatic organ or site (IIE)
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Clinical Stage 3
of Hodgkin and Non Hodgkin Lymphoma |
Involvement of 2 or more LN regions on the same side of the diaphragm alone (II) or with involvement of limited contiguous extralymphatic organ or site (IIE)
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Clinical Stage 4
of Hodgkin and Non Hodgkin Lymphoma |
Multiple of disseminated foci of involvement of one or more extralymphatic organs or tissues with/without lymphatic involvement.
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Nodular Sclerosis
Hodgkin's Lymphoma Morphologic Features |
- Frequent lacunar/R-S cells
- Background infiltrate of lymphs, eosinophils, plasma cells, macrophages - FIBROUS BANDS divide into nodules - CAPSULAR FIBROSIS |
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Nodular Sclerosis
Hodgkin's Lymphoma Clinical Features |
- 70% of Hodgkin Lymphomas
- Stage I/II most common - MEDIASTINAL INVOVLEMENT - FEMALES >> males - Most patients young adults |
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Mixed Cellularity
Hodgkin's Lymphoma Morphologic Features |
- Frequent mononuclear and R-S cells
- MIXED INFLAMMATORY BACKGROUND infiltrate with lymphs, eosinophils, plasma cells, macrophages, granulomas - DIFFUSE GROWTH, lacks nodules and fibrosis |
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Mixed Cellularity
Hodgkin's Lymphoma Clinical Features |
- 20% of Hodgkin Lymphomas
- >50% present at Stage III/IV - Males>>Females - Biphasic incidence: young adults and ADULTS > 55 YEARS |
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Lymphocyte Rich
Hodgkin's Lymphoma Morphologic Features |
- Frequent R-S cells in a BACKGROUND OF SMALL, REACTIVE LYMPHOCYTES
- lacks inflammatory infiltrates - Nodular or diffuse growth without fibrosis |
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Lymphocyte Rich
Hodgkin's Lymphoma Clinical Features |
- 5% of Hodgkin Lymphoma
- Stage I/II most common - Young males with cervical or axillary nodes - Mediastinal disease uncommon |
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Lymphocyte Depleted
Hodgkin's Lymphoma Morphologic Features |
- SHEETS OF R-S CELLS
- TUMOR NECROSIS - Inflammatory backgroun less conspicuous (mimics large cell lymphoma) - Diffuse growth with fibrosis |
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Lymphocyte Depleted
Hodgkin's Lymphoma Clinical Features |
- 5% of Hodgkin Lymphoma
- Older male patients with disseminated disease - Also seen in HIV/AIDS AND DEVELOPING COUNTRIES |
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What do the A and B of Hodgkin grading stand for?
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All stages are further divided on the basis of the absence (A) or presence (B) of the following systemic symptoms: significant fever, night sweats, or unexplained weight loss of > 10% of normal body weight.
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