WBC Pathology Flashcards
(49 cards)
Follicular Lymphoma
Differentiated from follicular hyperplasia by:
Disrupted architecture
Lack of a mantle zone w/ polarity
No dark/light zones w/ tangible body macros
Reverse bcl2 staining
Paracortical hyperplasia
Enhanced growth of the T-cell region due to stimulation via infection, drugs (Dilantin), and even dermatopathic lymphadenopathy
-Must correlate w/ clinical findings and TCR rearrangement studies
Sinus histiocytosis
Numerous macrophages within the lymph sinuses thought to be the response to malignant cells
-Will be prominent in nodes draining cancer
Nonspecific lymphadenitis
Follicular hyperplasia that occurs due to the drainage of infections; results in large, tender nodes
-Can be acute or chronic; common in kids
Precursor-B cell ALL
Clinical: Abrupt stormy onset, BONE PAIN (marrow involvement), possible CNS symptoms
Immunophenotype: CD19, CD22, CD10 (+), TdT (+)
Cytogenetics: Can involve t(12;21) mutating ETV6 and RUNX1 genes or t(9;22) mutating BCR-ABL
Treatment: Aggressive chemo including CNS prophylaxis
(Very successful in children; adults can sometimes not handle the chemo)
*Monitor to ensure there is no detection of MDR
Precursor T-cell ALL
Clinical: Abrupt stormy onset, Bone pain, CNS sx, MEDIASTINAL MASS
Diagnosis: MPO(-), disruption of normal architecture
*CD34, TdT, CD1a, CD2, CD5, CD7 (+)
*NOTCH1 mutations seen in 70%
Prognosis: Aggressive chemo and CNS prophylaxis
*Detection of MRD is assoc. w/ bad outcome
CLL/ASL
Clinical: Generalized lymhadenopathy w/ hypogammaglobulinemia (infxns)
Diagnosis: Smudge cells on PS, Increased small and mature lymphs that are hyperclumped,
(SLL)-lymphoid aggregates in BM and white/red pulp in spleen/liver
* Pale areas on lymph node can be indicative * CD19, CD5, CD23, CD20-dim, surface light chain restricted-dim
Prognosis=> Most pts. die of other problems
**Progression to Richter Syndrome possible
Follicular Lymphoma
Clinical: Painless lymphadenopathy, mimics normal architecture
Diagnosis: LN has a nodular pattern w/ lack of an asymmetric mantle zone; liver shows “portal tracks”
* CD10, 19, 20, surface light chain restriction * t(14;18) =>> bcl2 and bcl6 overexpression
Prognosis: Chemotherapy is ineffective but disease is indolent
–transformation to DLBCL occurs later
Bcl2
Inhibits apoptosis via the mitochondrial pathway
-Normally shut off to allow for normal lymphocyte maturation
Bcl6
Encodes for a DNA zinc finger that is normally required for normal germinal center regulation
Mantle Cell Lymphoma
Clinical: Painless lymphadenopathy, lymphomatoid polyposis, BM involvement
Diagnosis: CD5, CD19, CD20, BRIGHT surface light chain
* t(11;14) =>>Increased cyclin D1 expression and increased G1-S phase transition * Tumor cells can resemble that of normal mantle cells
Prognosis: Retuximab and Chemo; possible BM transplant in adolescents
*Most pts. relapse or die of organ failure in 3 yrs
Marginal Zone Lymphoma (nothing special)
Clinical: Assoc. w/ hyperinflammatory states (Hashimotos, Sjogrens); can regress if these states are controlled; cells can resemble normal marginal zone cells
Diagnosis: Pleomorphic population of plasmacytoid and monocytoid B-cells w/ destructive infiltration of host tissue
**Starts off as a reactive polyclonal reaction to inflammation, acquires mutations or t(11;18) to express MALT1 or BCL10 that will not respond to extrinsic signaling =»lymphoproliferation
Causes of lymphadenopathy
AI disorders- Sjogrens, SLE
Iatrogenic- Drugs, silicone
Infection
Malignancy
Sarcoidosis
Dermatopathic lymphadenopathy
Lymphoplasmocytic Leukemia
Resembles SLL only most neoplastic cells are plasma cells; secretes monoclonal IgM =»Waldenstrom’s Macroglobulinemia
-Cryoglobulinemai, visual/neurologic symptoms
- Involves a mutation in the MYD88 gene
- promotes the growth and survival of tumor cells
-Plasmapharesis alleviates symptoms
Hairy Cell Leukemia
Clinical: Monocytopenia, splenomegaly; *assoc. w/ mutations in BRAF
Diagnosis: Diffuse, fried eggs in the BM; dry tap of BM; red pulp involvement w/ obliteration of the white pulp
*CD11c/CD25/CD103 (+) =»CHARACTERISTIC
Prognosis: Indolent course; use chemo w/ BRAF inhibitors
Multiple Myeloma
Pathophysiology: MUST CONTAIN
- M-protein in blood/urine
- Monoclonal plasma cells
- End organ damage
Clinical: Weakness (anemia), recurrent infxn, polyuria (hypercalcemia), renal failure (BJ protein), amyloidosis
* Labs: BJ protein in urine; increased IgG or IgA; anemia; Rouleux and "Blue smear" on peripheral blood slide
- Bony, lytic lesions on x-rays of bone
- Caused by translocations involving IgH, del17p, and rearrangements involving MYC
- Increased IL-6 also increases the activity of osteoclasts
Treatment: Chemo and stem cell transplant may prolong life but not cure
MGUS
“Monoclonal Gammopathy of Unknown Significance”
-Most common cause of monoclonal gammopathy that must be monitored (BJ protein and serum protein M levels) to ensure it doesn’t progress to MM
Plasmacytoma
Soft tissue clonal plasma cell masses that commonly collect on the spine (bone site) or lung/oropharynx (soft tissue site)
-Can progress to MM; treat w/ localized radiation
Diffuse Large B-Cell Lymphoma
Clinical: B-grade symptoms w/ rapidly forming mass; BM involvement later
Diagnosis: Diffuse disruption of BM architecture by large lymphocytes; mitotically active and have indistinct cell borders
*No pathognomic cell markers, just CD10, 19, surface light chain
*Can have bcl2, bcl6, or myc8 gene rearrangement
=»DLBCL w/ two of these is much more aggressive
Treatment: R-CHOP works pretty well; fatal if untreated tho
*Can occur in IC pts. where it is assoc. w/ EBV infection
Burkitt Lymphoma
Clinical: 30% of childhood NHL; usually extranodal w/ mandibular mass and abdominal masses
=»can lead to Tumor Lysis Syndrome
*Assoc. w/ EBV infections in endemic areas
Diagnosis: “Starry Sky” appearance of BM (macros eating dead cells); infiltrate of medium sized cells w/ basophilic cytoplasm; high mitotic activity
- typically has translocation of MYC protein allowing for increased glycolysis by the tumor
* Is bcl2, CD34, and Tdt (-) because the cells are MATURE
***ASSOC. W/ t (8;14)
Treatment: Responds well to systemic chemotherapy as well as intrathecal administration
Tumor Lysis Syndrome
Rapid cell turnover and death causes the release of uric acid, K+, and Ca2+
=»Medical emergency requiring hydration, binding of electrolytes, and hemodialysis
Peripheral T-Cell Lymphoma
Clinical: General lymphadenopathy, eosinophilia
Diagnosis: Large sized malignant cells w/ paracortical effacement; CD34 and TdT (-)
Prognosis: Not good
Anaplastic Large Cell Lymphoma
Clinical: Extranodal infiltrate w/ BM involvement
*Involves ALK translocation which can be stained w/ immunohistochemistry
Diagnosis: *Hallmark= Large anaplastic cells w/ horseshoe appearing nucleus
*Neoplastic cells congregate around sinuses
Prognosis: Favorable unless you are adult and ALK (-)
Adult T-cell Leukemia
Clinical: T-cells infected w/ HTLV-1 that presents as generalized lymphadenopathy, skin lesions, and lymphocytosis
*Skin lesions only=favorable prognosis
Diagnosis: Cloverleaf cells; ^**CD4(+)/CD7(-); HTLV-1 (+)
