WBC disorders Flashcards
1
Q
Acute Leukemia
A
- neoplastic proliferation of blasts (> 20% blasts in BM)
- increased blasts “crowd out” normal hematopoiesis–> anemia, bleeding, infection
- acute onset
- blasts leak out into blood (large, immature cells with punched out nucleoli on blood smear)
- divided into ALL(TdT+) and AML (MPO+)
- high WBC
- younger population
2
Q
B-ALL
A
- B cell Acute Lymphoblastic Leukemia
- neoplastic proliferation of lymphoblasts
- most common form of childhood leukemia
- TdT (+)
- CD10, 19,20
- good prognosis for kids
3
Q
T-ALL
A
- T-cell Acute Lymphoblastic Leukemia/Lymphoma
- TdT (+)
- CD2-8
- T for teenagers and thymic mass (mediastinal adenopathy)
4
Q
chronic leukemia
A
- neoplastic proliferation of mature lymphocytes
- usually insidious onset; adults
- CLL, Hairy cell leukemia, ATLL, mycosis fungoides
5
Q
CLL
A
- Chronic Lymphocytic Leukemia
- lymphocytosis (hence the name! remember it’s a proliferation of mature B cells)
- involvement of LNs = small lymphocytic lymphoma (SLL)
- widespread lymphadenopathy + liver and spleen enlargement (unlike diffuse large B cell lymphoma)
- neoplastic proliferation of Naive B cells – antibody production is all screwed up –> hypogammaglobulinemia and autoimmune hemolytic anemia
- CD20 and CD5 (normally expressed on T cells)
- older adults; most common neoplasm overall
- smudge cells on blood smear
- Richter transformation: transform to diffuse large B-cell lymphoma (enlarging LN or spleen)
6
Q
Hairy Cell Leukemia
A
- neoplastic proliferation of mature B cells characterized by hairy cytoplasmic processes
- TRAP and TAP: TRAP stain, get trapped in spleen (no lymphadenopathy), “dry tap” (marrow fibrosis), trapped in hair
7
Q
ATLL
A
- Adult T Cell Leukemia
- neoplasm of mature T cell
- associated with HTLV-1 infection (Japan and Caribbean)
- rash
- hypercalcemia (lytic punched out bone lesions)
8
Q
Mycoses Fungoides
A
- neoplastic proliferatrion of mature CD4+ T cells that infiltrate skin –> skin rash, plaques and nodules
- Sezary cells: lymphocytes with cerebriform nuclei
Sezary and Mycoses Fungoides are very fancy
9
Q
myeloproliferative disorders
A
- neoplastic proliferation of mature cells of myeloid lineage (the mature version of AML)
- CML, PV, ET, myelofibrosis
- late adulthood
- high WBC with hypercellular BM (cells of all myeloid lineages are increased but disease defined by the predominant type)
- increased risk for hyupericemia and gout (due to increased cell turnover)
- progression to myelofibrosis (“burn out”) or AML (“blast out”)
10
Q
CML
A
- Chronic Myeloid Leukemia
- neoplastic proliferation of myeloblasts (neutrophils, eosinophils and basophils)
- Philly Chromo t(9;22) —BCR-ABL fusion protein (Philly CreaML cheese)
- can transform to AML or ALL (“blast crisis”) since mutation is in pluripotent stem cell
- low LAP as a result of low activity in mature granulocytes (vs. leukemoid rxn)
- responds to imatinib (blocks tyr kinase actvity of fusion protein)
11
Q
polycythemia vera
A
- neoplastic proliferation of mature myeloid cells, esp RBCs
- hematocrit > 60%
- JAK2 kinase mutation
- hyperviscous blood: blurry vision and HA, venous thrombosis (Budd-Chiari)
- intense itching after hot shower
- hyperuricemia and gout (due to rapid cell turnover)
- distinguished from reactive polycythemia by low EPO and normal SaO2
12
Q
AML
A
- acute myeloid leukemia
- myeloblasts characterized by MPO and Auer rods
- subtypes include APL, AMoL, AMegL
- may arise from pre-existing myeloproliferative disorders
- risk factors: exposure to alkylating agents, chemo, radiation
13
Q
APL
A
- Acute Promyelocytic Leukemia/ M3 (subtype of AML)
- MPO + Auer rods
- t(15;17) —- translocation of RAR –> RAR disruption blocks maturation and promyelocytes/blasts accumulate
- tx: all-trans RA (vit A)
- DIC: high concentration of Auer rods released upon chemotx
14
Q
ET
A
- Essential Thrombocytopenia
- neoplastic proliferation of mature myeloid cells, esp platelets
- JAK2 mutation
- increased risk of bleeding (dysfunctional platelets) and/or thrombosis (hyperactive platelets)
- throbbing, burning pain in extremities caused by clotting of arterioles
15
Q
Myelofibrosis
A
- neoplastic proliferation of mature myeloid cells, esp megakaryocytes
- megakaryocytes produce PDGF –> marrow fibrosis
- splenomegaly due extramedually hematopoeisis
- tear-drop RBCs (RBCs squeezed out of fibrotic marrow)