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White Blood Cell Disorders
Terms in this set (23)
Proliferation of primitive hematopoietic cells in the bone marrow (i.e blasts). These "blasts" result in a increased WBC count. They also crowd out normal hematopoiesis resulting in symptoms of anemia, thrombocytopenia, and neutropenia.
Can be Acute lymphoblastic Leukemia or Acute Myeloid Leukemia.
Acute Lymphoblastic Leukemia
Accumulation of lymphoblasts in the bone marrow. + for TdT (AML is negative for TdT). It is the most common malignancy of childhood in the US. Often seen with a simultaneous trisomy of chromosomes 4,10 and 17.
B Cell: Markers include CD19 CD20 and CD22.
- t(9;22) translocation is associated with a bad prognosis
- t(12;21) is associated with a good prognosis.
- t(4;11) occurs in 2-3% of cases of ALL most commonly with those in infants.
T Cell: Markers include CD2 CD5 and CD7. Classical presentation is an adolescent male with mediastinal mass or peripheral lymphadenopathy and a high WBC.
A subtype of ALL with the worst prognosis.
Morphology: Large size, oval to round nucleus, multiple prominent nuclei, moderately abundant cytoplasm with intense basophilia and clearly demarcated vacuoles.
Markers include CD19 CD 20 and CD22 w/ IgM .
- t(8;14) translocation.
Chronic Lymphocytic Leukemia
Neoplastic proliferation of mature lymphocytes which appear in the peripheral blood, bone marrow and lymph nodes. Most common leukemia in the US and a disease of older adults.
Markers: CD 19, CD 20, CD 23 and CD 5. IgM also on surface.
Prognosis: Usually good, most px are asymptomatic. Disease originating from naive B cells will have a worse prognosis and are marked by CD 38. Disease due to a memory B cell which has undergone somatic hyper mutation will have a better outcome.
- Hypogammaglobulinemia: low immunoglobin levels due to only specific types of antibodies being produced (if any are produced). Leads to Infection, #1 cause of death.
- Autoimmune Hemolytic Anemia: Bad AB attack self
- Transformation to large B cell lymphoma (marked by enlarging lymph node or spleen)
Hairy Cell Leukemia
HCL is a form of B cell chronic leukemia. Characterized by a insidious onset, massive splenomegaly without lymphadenopathy.
Cells have numerous hair like projections around the periphery of the cytoplasm. The cytoplasm is abundant and pale blue and appears delicate. Cells are positive for Tartrate resistant acid phosphatase. (TRAP)
Small Lymphocytic Lymphoma
Non Hodgkin Lymphoma, Diffuse, Small B Cell
Disease effects older adults and presents with multisite lymphadenopathy and bone marrow involvement.
Markers include CD 19, CD20 and CD23 as well as CD5. Monoclonality.
Non Hodgkin Lymphoma, Follicular, Small B Cells (centrocytes and centroblasts). The neoplastic follicles are "back to back."
Very common NH lymphoma, median age of diagnosis is 59, multisite lymphadenopathy with frequent marrow involvement. Slowly progressing disease.
Markers include CD19, CD20, CD 22, CD 10 as well as a single immunoglobulin light chain type, kappa or lamda, indicating monoclonality. CHARACTERISTIC t(14;18) translocation. Results in overepression of BCL-2 which inhibits apoptosis.
Mantle Cell Lymphoma
Non Hodgkin Lymphoma; Diffuse or Follicular; small B cells
- neoplastic proliferation into the mantle zone
Markers: Monoclonal kappa or lambda light chain with CD19 CD 20, possibly CD5 but NO CD23. Driven by a t(11;14) translocation which results in a overexpression of cyclin D1.
Non Hodgkin Lymphoma; Diffuse; Intermediate B Cells
- Cells have fine nuclear chromatin, prominent multiple nucleoli,
narrow rims of purple cytoplasm.
Can present as a extranodal mass and occurs commonly in children. Histology shows a high mitotic index and a "starry sky" appearance.
Characteristic t(8;14) translocation which results in overexpression of c-myc
Diffuse Large B Cell Lymphoma
Non Hodgkin Lymphoma, Diffuse, Large B cells
Most common form of NHL and occurs in all age groups. The cells grow in sheets and it is clinically aggressive.
50% cure rate with aggressive therapy CHOP-R
Hodgkin vs Non Hodgkin Lymphoma
NHL effects the elderly more while Hodgkin lymphoma is a disease of young adults with a peak incidence in the second, third, and fourth decades of life.
NHL neoplastic cells are lymphoid cells while in HL they are Reed-Sternberg cells.
NHL spread is diffuse and commonly presents extranodally. HL, however, always presents within lymph nodes and spreads contiguously.
In NHL, the mass is mostly lymphoid cells while in HL, the mass is mostly inflammatory cells with some malignant RS Cells.
Nodular Lymphocyte Predominant Hodgkin Lymphoma (NLPHD)
Hodgkin Lymphoma. (approx 5% of all cases)
- low grade B cell lymphoma
- younger age groups, good prognosis.
- Nodules are populated by a mixture of small round lymphocytes
and reactive histocytes with LP cells.
Differs from classical Hodgkin lymphoma because we have LP or Popcorn cells instead of Reed-Sternberg cells.
LP cells have a large folded to lobulated delicate nucleus with an open chromatin pattern and small nucleoli with a smalla mount of ill-defined cytoplasm.
LP Cell Markers: CD 20 and CD45. Negative for CD 15 and CD 30 (classical RS cells are +)
Hodgkin Lymphoma (approx 60%, most common type)
Most px present with an enlarging cervical or mediastinal lymph node. Px are often stage 2 at presentation and have a very good prognosis.
Lymph node is divided by connective tissue septae. The RS cells are present in lake like spaces and are referred to as lacunar cells. These cells are present in a inflammatory background composed of lymphocytes, eosinophils, neutrophils, histocytes and plasma cells.
Hodgkin Lymphoma (30% of cases)
RS cells in a varied inflammatory background with abundant eosinophils
Hodgkin Lymphoma (rare)
Similar in morphology to NLPHD BUT has normal RS cells.
Best prognosis compared to other Hodgkin Lymphoma
Hodgkin Lymphoma (rare)
Few lymphocyte background cells and many RS cells.
Most aggressive of all types seen often in elderly and HIV positive individuals.
Acute Myeloid Leukemia
Neoplastic proliferation of blast cells of myeloid lineage. Dx occurs when we have greater than 20% of blasts in the bone morrow or peripheral blood.
Accumulation of blasts in the bone marrow results in interference of normal blood cell production and symptoms of anemia, neutropenia and thrombocytopenia.
Myeloblasts can be distinguished from lymphoblasts by + staining for myeloperoxidase. Crystal aggregates of MPO can be seen as auer rods and are indicative of a diagnosis for AML or MDS.
Cytogenics: Px with de novo AML often have balanced translocations while px with hx of MDS or prior chemo/radio therapy will be more likely to have monosomy and deletions of chromosomes 5 & 7
A group of closely related clonal hematopoietic disorders characterized by a hyper or hypo cellular marrow and impaired morphology and maturation and increased degree of apoptosis. Mostly effects the elderly and is discovered accidentally.
Dx: increased blasts but less than 20% and presence of ringed sideroblasts.
Chronic Myeloid Leukemia
Myeloproliferative Disorder of granulocytes, especially basophils. Occurs mostly in late adulthood.
Characteristic t(9;22) mutation resulting in BCR-ABL fusion which results in increased tyrosine kinase activity.
Myeloproliferative Disorder, especially effecting RBC.
Associated with a JAK2 Kinase mutation
Myeloproliferative Disorder, especially effecting platelets.
Symptoms might be a result in increased bleeding or increased thrombosis.
Associated with a JAK2 Kinase Mutation
Myeloproliferative Disorder, especially effecting megakaryocytes
Megakaryocytes produce excess platelet derived growth factor resulting in bone marrow fibrosis and extramedullary hematopoiesis in the spleen. This results in splenomegaly.
Tear drop RBC, nucleated RBC and immature granulocytes will be present due to immature cells leaving spleen.
Plasma Cell (Multiple) Myeloma
A disseminated plasma cell malignancy with a peak incidence in the seventh and eighth decades of life.
1: Monoclonal protein in the serum and or urine
2: Bone marrow clonal plasma cells or plasmacytoma
3: Related organ or tissue impairment.
a. Renal insufficiency due to renal tubule damage secondary to the reabsorption or percipitation of light chains.
b. Osteolytic bone lesions located in areas of active red marrow including spine, ribs, skull, and pelivs. Increased risk for fracture and hypercalcemia.
c. Anemia due to suppression of erythropoiesis due to the increase in amount of plasma cells in bone marrow.
e. Amyloidosis due to deposition of light chain or light chain fragments in the tissues producing organ dysfunction.
f. Increased risk of infection due to the large amounts of a specific monoclonal antibody being made. This results in lack of antigenic diversity (most common cause of death)
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