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Pathology 1 - Block 3 - Part 2

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davidwurbel7's version from 2016-03-31 19:34

White Blood Cells and Lymph Nodes 2

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Neoplastic disease that results from uncontrolled proliferation and incomplete maturation of hematopoietic precursors in bone marrow. These cells would crowd the marrow to displace the remaining “normal” cells. The neoplastic blood cells then enter into circulation and may involve other reticuloendothelial organs The cells are usually immature cell (blasts)Leukemia
Neoplastic disease in which proliferations arising as discrete tissue massesLymphomas
Neoplastic disease that is composed of tumors containing terminally differentiated B - cells. Arise mostly from bone marrow, with rare involvement of lymph nodesPlasma Cell Neoplasms
Acute Leukemia mostly seen in Pediatric age group. Peak age is between 2 and 5 yearsAcute Lymphoblastic Leukemia (ALL)
Acute Leukemia mostly seen in older age group (40 to 60) but can be seen in all age groupsAcute Myelogenous Leukemia (AML)
Blasts account for more than 20% in the bone marrow is the diagnostic criteria for this(Lymphoblastic???) Leukemia
Type of ALL that is seen to affect older child (adolescent)T-Cell Acute Lymphoblastic Leukemia (T-ALL)
NOTCH1 mutation in 70% of cases is associated with this conditionT-Cell Acute Lymphoblastic Leukemia (T-ALL)
PAX5 mutation, or t(12;21) involving ETV6/RUNX1, which promote maturation arrest is associated with this conditionB-Cell Acute Lymphoblastic Leukemia (B-ALL)
B-cell ALL can be associated with this translocationt(12:21) Translocation
A fusion of a gene. Seen in mostly in adults, carrying poor prognosis. If seen in children, they fare bettert(9; 22) B-Cell Acute Lymphoblastic Leukemia (ALL)
Lab Investigations - anemia, neutropenia and thrombocytopenia. WBC counts: elevated. Hb: decreased. Platelet count: decreased. Peripheral smear: circulating leukemic blast cells. Bone marrow biopsy presence of leukemic blast cells (immature cells of lymphoid origin) more than 20% blastsAcute Lymphoblastic Leukemia (ALL)
May contain 1 to 2 nucleoli, show dense chromatinLymphoblast
May contain 2 to 5 nucleoliMyeloblasts
Lab Investigations - anemia, neutropenia and thrombocytopenia. WBC counts: elevated. Hb: decreased. Platelet count: decreased. Peripheral smear: circulating leukemic blast cells. Bone marrow biopsy presence of leukemic blast cells (immature cells of lymphoid origin) more than 20% blasts. Serum lactate dehydrogenase (LDH) increased. Serum uric acid increased. Chest X-ray shows thymic enlargement and mediastinal lymph node involvementT- Cell Acute Lymphoblastic Leukemia (T-ALL)
Lab Investigations - anemia, neutropenia and thrombocytopenia. WBC counts: elevated. Hb: decreased. Platelet count: decreased. Peripheral smear: circulating leukemic blast cells. Bone marrow biopsy presence of leukemic blast cells (immature cells of lymphoid origin) more than 20% blasts. Serum lactate dehydrogenase (LDH) increased. Serum uric acid increased. Chest X-ray shows no thymic enlargement and no mediastinal lymph node involvementB-Cell Acute Lymphoblastic Leukemia (B-ALL)
CNS & testes are regarded as these type of sites for ALLSanctuary Sites
Clinical features include anemia, neutropenia and thrombocytopenia. Typical features include, abrupt stormy onset, bone marrow depression, bone pain and tenderness, generalized lymphadenopathy, CNS manifestationsAcute Lymphoblastic Leukemia (ALL)
Clinical features include anemia, neutropenia and thrombocytopenia. Typical features include, abrupt stormy onset, bone marrow depression, bone pain and tenderness, generalized lymphadenopathy, CNS manifestations, anterior mediastinal lymphadenopathy involvementT-Cell Acute Lymphoblastic Leukemia (T-ALL)
Tumor of hematopoietic progenitors caused by acquired oncogenic mutations that impede differentiation leading to accumulation of immature myeloid blasts in bone marrowAcute Myelogenous Leukenia (AML)
Translocation t(15;17) is associated with this AMLAML M3
Inversion of 16 is associated with this AMLAML M4
Translocation t(8;21) is associated with this AMLAML M2
Minimally differentiated. Myeloblast marker negativeM0
AML without differentiation. No maturation beyond the myeloblast stageM1
AML with differentiation. Maturation positiveM2
Acute Promyelocytic Leukemia. Promyeloctyes and early stages seen in bloodM3
Acute Myelomonocytic Leukemia. Presence of monocyte ligeage seen in bloodM4
Acute Monocytic Leukemia. Monoblasts seen in bloodM5
Acute Eryrtholeukemia. Erythroid and myeloblasts seen in bloodM6
Acute Megakaryocytic Leukemia. Megakaryocytic lineage seen in bloodM7
Young adult, males. numerous Auer rods present. bleeding tendencies. risk of DIC. features of BM failure. association with t(15; 17)AML: M3
MDS that becomes malignant becomes this conditionAML
Any age group. extramedullary features include: cutaneous and gingival infiltration, CNS involvement, bleeding tendency, positive for non-specific esterase (NSE). Poor prognosisAcute Monocytic Leukemia (M5)
Lab investigations: Total white cell count: increased, sometimes may be normal or *decreased. Anemia, thrombocytopenia. Peripheral blood smear: presence of atypical myeloid cells, circulating ‘blasts’. Bone marrow: >20% blasts. Cytogenetic studies. fever, splenomegaly, hepatomegaly, lymphadenopathy, sternal tenderness, evidence of infection, hemorrhage, easy bruising, petechiae, epistaxis, gingival bleeding, conjunctival hemorrhages, and prolonged bleeding from skin injuries reflect thrombocytopenia and are frequent early manifestations of the disease. Gastrointestinal, intrapulmonary or intracranial hemorrhages, DICAcute Myeloid Leukemia M3 (AML-M3)
Lab investigations: Total white cell count: increased, sometimes may be normal or *decreased. Anemia, thrombocytopenia. Peripheral blood smear: presence of atypical myeloid cells, circulating ‘blasts’. Bone marrow: >20% blasts. Cytogenetic studies. fever, splenomegaly, hepatomegaly, lymphadenopathy, sternal tenderness, evidence of infection, hemorrhage, easy bruising, petechiae, epistaxis, gingival bleeding, conjunctival hemorrhages, and prolonged bleeding from skin injuries reflect thrombocytopenia and are frequent early manifestations of the disease. Gingival infiltration, meningeal involvementAcute Myeloid Leukemia M5 (AML-M5)
Lab investigations: Total white cell count: increased, sometimes may be normal or *decreased. Anemia, thrombocytopenia. Peripheral blood smear: presence of atypical myeloid cells, circulating ‘blasts’. Bone marrow: >20% blasts. Cytogenetic studies. fever, splenomegaly, hepatomegaly, lymphadenopathy, sternal tenderness, evidence of infection, hemorrhage, easy bruising, petechiae, epistaxis, gingival bleeding, conjunctival hemorrhages, and prolonged bleeding from skin injuries reflect thrombocytopenia and are frequent early manifestations of the disease. Increased association with Down syndromeAcute Myeloid Leukemia M7 (AML-M7)
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White Blood Cells and Lymph Nodes 3

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Lab Investigations - Hb: anemia very late in the disease . White cell count: markedly elevated. Peripheral blood smear: lymphocytosis, ‘smudge cells’. Bone marrow: involved by neoplastic B cells. Immunoglobulin: Low level of surface Ig expressed. Markers: express pan B cell markers (CD19, CD20) CD5, and CD23 are positiveChronic Lymphocytic Leukemia (CLL)
Clinical features - usually asymptomatic (incidental). non specific symptoms: tiredness, weight loss. generalized lymphadenopathy, hepatosplenomegaly. hypogammaglobulinemia, which predisposes to infections. 10 to 15% have antibodies which are directed against rbc and platelets: autoimmune hemolytic anemia (AIHA) or thrombocytopeniaChronic Lymphocytic Leukemia (CLL)
Rapidly enlarging mass within lymph node/spleen which is a “diffuse large B cell lymphoma”. Survival is less than a year in both transformations. Acquisition of additional mutation is the likely cause for transformation. Transformation is associated with poor prognosisRichter Syndrome
Rare chronic lympho-proliferative disorder of B lymphocytes morphologically characterized by prominent cytoplasmic hair-like projections. Incidence: middle age, median age of 55 years, more common in males (5:1 male to female ratio). Pathogenesis: activating point mutation in BRAF seen in 90%. Overexpression of cyclin D1 (cell cycle regulator)Hairy Cell Leukemia
Lab Investigations - Hb: decreased (anemia) Total WBC: decreased Platelet count: decreased “pancytopenia”. Peripheral blood smear: neutropenia, presence of hairy cells. Bone Marrow: “dry tap” bone marrow is involved, where they are enmeshed in extra cellular matrix bone marrow biopsy indicated leukemic cells have hair-like structure on surface of cytoplasmHairy Cell Leukemia
Chronic Lymphocytic Leukemia (CLL) transforms into this with the accumulation of more mutationsDiffuse Large B Cell Lymphoma
Lympho-proliferative disorder seen in adults, associated with HTLV 1 infection, long latency, Japan, West Africa, Caribbean. Transmission: blood, sexual, transplacental, breast milk (low risk)Adult T-Cell Leukemia/Lymphoma
Pathogenesis tropism for CD4+ T cells. HTLV-1 has a region referred to as TAX. Inactivates cell cycle inhibitor p16/INK4a and activates cyclin D1. TAX activates NF-kB. TAX gene inhibits p53Adult T-Cell Leukemia/Lymphoma
Labs - CBC: leukocytosis. Peripheral blood smear: lymphocytosis, abnormal lymphoid cells described as ‘flower cells’ seen. Biochemical: Hypercalcemia. Skin Biopsy: T-cell markers positive (CD4). Serum: HTLV-1. Radiology: ‘lytic’ bone lesionsAdult T-Cell Leukemia/Lymphoma
Clinical features include rapidly progressive disease, aggressive lymphadenopathy. hepato-splenomegaly. skin infiltration (lesions). hypercalcemia, and lytic bone lesions. high mortalityAdult T Cell Leukemia/Lymphoma
Immunophenotype - mature T cell markers: CD2, CD3, CD4, on skin biopsies. Clinical features - progresses from patch to plaque to tumor phase but patients may simultaneously have more than one type of lesion. indolent diseaseMycosis Fungoides
Histology shows infiltration of epidermis and dermis by neoplastic T cells typically a “band-like and patchy infiltrate” in upper dermis of atypical lymphocytes may spread to lymph nodesMycosis Fungoides
Collections of neoplastic lymphocytes in the epidermis that cluster that is associated with Mycosis FungoidesPautrier Abscess
Characterized by tumor dissemination to other organs and spill over into peripheral blood (Mycosis fungoides with a leukemic phase). Cells are characterized by cerebriform nucleiSézary Syndrome
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