Pathoma WBC Flashcards
Neutropenia
2 causes
Treatment
Dec. circulating neutrophils
Causes:
1. Drug toxicity (damage stem cells)
2. Severe infection (neutrophils move into tissue)
Treatment: GM-CSF or G-CSF may boost granulocyte production.
Lymphopenia
4 causes
Dec. circulating lymphocytes Causes: 1. Immunodeficiency (DiGeorge, HIV) 2. High cortisol state (corticosteroids or Cushings) 3. Autoimmune destruction (SLE) 4. Whole body radiation
Neutrophilic leukocytosis
2 causes
Inc. circulating neutrophils
- Bacterial infection or tissue necrosis induces release of marginated pool of BM neutrophils including immature forms (Left shift, dec CD16)
- High cortisol state impairs adhesion, release marginated pool
Monocytosis
2 causes
Inc circulating monocytes.
Causes:
1. chronic inflammation (autoimmune and infectious)
2. malignancy
Eosinophilia
3 causes
Inc. circulating eosinophils
- allergic reactions (type I hypersensitivity)
- parasitic infections
- Hodgkin Lymphoma (inc IL-5 by RS)
Basophilia
Inc circulating basophils, seen in chronic myeloid leukemia
Lymphocytic leukocytosis
2 Causes
Inc. circulating lymphocytes
- Viral infections (CD8+ hyperplasia)
- Bordetella pertussis infection - bacteria prevent circulating lymphocytes from elaving blood to enter lymph nodes (so that the lymphocytes can’t get educated)
Infectious Mononucleosis
Presentation and Test
EBV affects CD8+ T cells.
Effects:
1. Generalized LAD due to paracortex T cell hyperplasia
2. Splenomegaly (white pulp hyperplasia)
3. High WBC count with atypical lymphocytes (reactive CD8+ cells)
Monospot test for screening: detects IgM that cross react w horse or sheep RBC’s, specific for EBV. (If negative, could be CMV)
Acute leukemia: 3 clinical presentations plus an example of what the cells look like
anemia, thrombocytopenia, neutropenia
Large immature cells with punched out nucleoli.
B-ALL
Associations and gene
Most common type of ALL,
Tdt+ lymphoblasts with CD10, CD19, CD20, CD22
t(12;21) is good prognosis (kids)
t(9;22) is bad prognosis (adults)
T-ALL
Associations
Lymphoblasts from CD2-CD8, NO CD10
T’s: Thymic mass (mediastinum) and Teenagers.
–> Acute lymphoblastic Lymphoma
Acute promyelocytic leukemia (APL)
gene and pathology
AML-M3
t(15;17), RAR disruption blocks maturation and promyelocytes (blasts) accumulate.
Treat with ATRA
Acute monocytic leukemia
Association and gene
AML-M5
t(9;11), t(11;19), del(11q)
Proliferation of monoblasts, usually lack MPO
BLASTS INFILTRATE GUMS.
Acute megakaryoblastic anemia
Association
AML-M7
Proliferation of megakaryoblasts, lack MPO
ASSOCIATED W DOWN SYNDROME BEFORE AGE 5
Myelodysplastic syndrome
May lead to Acute myeloid Leukemia.
Present with cytopenias, hypercellular bone marrow, abnormal maturation of cells, and increased blasts.
Most pts die from infection or bleeding
Chronic Lymphocytic Leukemia
- associations
- 3 complications
CLL (“silly”)
Neoplastic proliferation of naive B cells (mature-appearing)
that coexpress CD5 and CD20 (CD5 is normally a T cell marker).
- SMUDGE CELLS
- CLL in lymph nodes = SLL (naive, “SLLY”)
Complications:
- Hypogammaglobulinemia - neoplastic cells do not become plasma cells.
- Autoimmune hemolytic anemia (dysfunction in Ig production)
- Transformation to diffuse large B cell lymphoma (RICHTER TRANSFORMATION) marked by enlarging lymph node or spleen.
Hairy Cell Leukemia
3 associations and a treatment if ya want!
A type of chronic leukemia
Neoplastic proliferation of mature B cells
1. TRAP+
2. Splenomegally = cells accumulate in Red Pulp
3. Bone Marrow fibrosis
*treat with 2-CDA (cladribine), an adenosine deaminase inhibitor)
Adult T-cell leukemia/lymphoma
ATLL - Neoplastic proliferation of mature CD4+ T cells
Associated with HTLV-1 (Japan and Caribbean)
Clinical Presentation
1. Rash (T cells love skin!)
2. Generalized LAD & Hepatosplenomegaly
3. lytic (punched out) lytic bone lesions with hypercalcemia
Mycosis Fungoides
2 associations, and basic pathology
Mycosis Fungoides is aka CUTANEOUS T CELL LYMPHOMA (CTCL)
- neoplastic proliferatio n of mature CD4+ T cells that infiltrate skin: skin plaques, nodules.
1. PAUTRIER MICROABSCESSES = on skin
2. SEZARY SYNDROME = in blood (sezary cells =cerebriform nuclei seen on blood smear)
Myeloproliferative disorders, 2 general complications
- Hyperuricemia or gout (high turnover of cells0
- progression to marrow fibrosis (burnt out or spent phase) or transformation to acute leukemia if differentiation is arrested.
Chronic Myeloid Leukemia
Neoplastic proliferation of mature myeloid cells, especially granulocytes and their precurors. Basophils are characteristically increased compared to an inflammatory reaction.
t(9;22)
Mutation in HSC
Treat w Gleevec (imatinib)
Polycythemia Vera
definition, mutation, 4 clinical symptoms, EPO levels
Neoplastic proliferation of mature myeloid cells, especially RBCs.
Jak2 kinase mutation.
Clinical symptoms:
1. Blurry vision and headache
2. Inc risk of venous thrombosis (i.e. Budd chair (thrombosis in hepatic vein leading to infarction of liver)
3. Flushed face due to congestion in blood vessels
4. Itching especially after bathing (histamine release from mast cells)
EPO levels are DECREASED.
Essential Thrombocythemia
ET
Neoplastic proliferation of mature myeloid cells, especially platelets.
JAK2 kinase mutation
Bleeding and/or thrombosis (nonfunctional platelets vs. many functional platelets)
No risk of hyperuricemia or Gout (no nuclei)
Myelofibrosis
key association, 3 clinical features
Neoplastic proliferation of mature myeloid cells, paticularly megakaryocytes
JAK2 kinase mutation
–> excess PDGF produced causing
BONE MARROW FIBROSIS:
Clinical features:
- splenomegally (extramedullary hematopoiesis)
- Leukoerythroblastic smear (tear drop RBCs damage from BM fibrosis, nucleated RBCs, immature granulocytes)
- Inc risk of infection, thrombosis, bleeding.
