Chapter 6: High yield White Blood Cell Disorders Flashcards
CD 34
hematopoietic stem cell
CD 16
Fc receptor. Decreased in immature neutrophils.
Left shift?
release immature neutrophils
Eosinophilia
Think Hodgkin Lymphoma.
Basophilia
Think chronic myeloid leukemia
Positive TdT nuclear staining means
Its a lymphoblast - so would be ALL. TdT is a DNA polymerase
Tdt+; CD10,19, and 20. t(12;21) [children] t(9;22) [adults]
B-ALL
TdT+; CD2-CD8 NO CD10
T-ALL. if present in teens as a mediastinal (thymic) mass - call acute lymphoblastic LYMPHOMA because the malignant cells form a mass.
MPO positive in cytoplasm means
Myeloblasts. will get crystal aggregates of MPO into AUER RODS
MPO +; t(15;17)
APL (Acute promyelocytic leukemia). retinoic acid receptor (17). Risk of DIC. Tx = all-trans-retinoic acid (vitamin A derivative)
Cytopenia; hypercellular bone marrow; increased blasts (but < 20%); prior exposure to alkylating agents or radiotherapy
Myelodysplastic syndrome
Neoplastic proliferation of blasts; defiend as the accumation of >20% blasts in the bone marrow
Acute Leukemia
Neoplastic proliferation of mature circulating lymphocytes; characterized by a high WBC count
Chronic Leukemia
Neoplastic proliferation of naïve B cells that co-express CD5 and CD20. Increased lymphocytes and smudge cells. Generalized lymphadenopathy.
Chronic Lymphocytic Leukemia (CLL); Complications = Hypogammaglobulinemia, Autoimmune hemolytic anemia, and transformation to DIFFUSE LARGE B-CELL LYMPHOMA (RICHTER TRANSFORMATION) Marked by enlarging lymph node or spleen
Neoplastic proliferation of mature B cell characterized by HAIRY CYTOPLASMIC PROCESSES. + tartrate-resistant acid phosphatase (TRAP). splenomegaly [RED PULP], dry tap on bone marrow
Hairy Cell leukemia
Tx of hairy cell leukemia
2-CDA (Cladribine) - an adenosine deaminase inhibitor
Neoplastic proliferation of mature CD4+ T cells. Associated with HTLV-1. Rash, generalized lymphadenopathy; hepatosplenomegaly; lytic bone lesions with hypercalcemia
Adult T-Cell leukemia/lymphoma (ATLL)
Neoplastic proliferation of mature CD4+ T cells. Pautrier microabscesses. Sezary Syndrome
Mycosis Fungoides
Neoplastic proliferation of mature myeloid cells, especially granulocytes (BASOPHILS). t(9:22) [generates BCR-ABL fusion protein with increased tyrosine kinase activity]. Negative leukocyte alkaline phosphatase (LAP) stain.
Chronic Myeloid Leukemia (CML). Can transform to AML (2/3) or ALL (1/3).
Tx of CML
Imatinib - blocks tyrosine kinase activity
Neoplastic proliferation of mature myeloid cells, especially RBC. Associated with JAK2 kinase mutation. Itching after bathing. EPO is decreased.
Polycythemia Vera. tx: phlebotomy. Second line = hydroxyurea
Neoplastic proliferation of mature myeloid cells, especially platelets. Associated with JAK2 kinase mutation.
Essential Thrombocythemia (ET)
Neoplastic proliferation of mature myeloid cells, especially megakaryocytes. Associated with Jak2 kinase mutation. Excess platelet-derived growth factor causing marrow fibrosis. Dacrocytes. Tear-drop RBC, nucleated RBC. Splenomegaly
Myelofibrosis
Rheumatoid arthritis and early stages of HIV infection show what area of lymph node hyperplasia
Follicular hyperplasia (B-cell region)
What region of the lymph node undergoes hyperplasia with viral infections (infectious mononucleosis)
Paracortex hyperplasia (T-cell region)
When do you see hyperplasia of sinus histiocytes in lymph nodes
nodes that are training tissue with cancer
Neoplastic proliferation of lymphoid cells that forms a mass. May arise in a lymph node or in extranodal tissue
Lymphoma
Neoplastic proliferation of small B cells (CD20). Late adult. painless lymphadenopathy. t(14:18)
Follicular Lymphoma. BCL2 on Chromosome 18. CAN PROGRESS TO DIFFUSE LARGE B-CELL LYMPHOMA - presents as an enlarging lymph node
Tx of Follicular lymphoma.
symptomatic only. Low-dose chemo or rituximab (anti-CD20 antibody)
Neoplastic proliferation of small B cells (CD20). T(11;14). Late adult, painless lymphadenopathy.
Mantel Cell lymphoma. Cyclin D1 gene on chromosome 11 Translocates to Ig heavy chain. Cyclin D1 promotes G1/S transition.
Tx of Mantel Cell lymphoma
Ibrutinib
Neoplastic proliferation of intermediate-sized B cells (CD20). Associated with EBV. extranodla mass in child/young adult. t(8;14) (c-myc). ‘starry-sky’ appearance
Burkitt Lymphoma
Neoplastic proliferation of large B cells (CD20) that grow diffusely in sheets. Late adult hood as an enlarging lymph node or extranodal mass.
Diffuse Large B-cell lymphoma.
tx: R-CHOP
Neoplastic proliferation of Reed-Sternberg cells (large B cell, multi lobed ‘owl eye’). CD15, CD30. Spread = contiguous
Hodgkin Lymphoma.
Malignant proliferation of plasma cells in the bone marrow. Bone pain with hypercalcemia (‘punched-out’); M-spike > 3; Rouleaux formation; AL amyloidosis; Bence Jones protein in urine.
Multiple Myeloma.
Neoplastic plasma cells activate the RANK receptor on osteoclast. see in vertebrae and skull.
CRAB Criteria for Multiple Myeloma
Calcium, Renal dysfunction, Anemia, Bone
M Spike <3g/Dl; no CRAB criteria;
Monoclonal Gammopathy of Undetermined Significance
B-cell Lymphoma with monoclonal IgM production. Mspike; Visual and neurological deficits; Bleeding. No lytic bone lesions.
Waldenstrom Macroglobulinemia Acute complications are treated with plasmapheresis
Birbeck (Tennis Racket) granules; CD1a; S100+
Langerhans cell histiocytosis
Malignant proliferation of Langerhans cells. Skin rash and cystic skeletal defects in an infant. Rapidly fatal.
Letterer-Siwe Disease
Benign proliferation of Langerhans cells in bone. Pathologic fracture in adolescent. Skin not involved.
Eosinophilic Granuloma
Malignant proliferation of Langerhans cells. Scalp rash; lytic skull defects; diabetes insipidus; exophthalmos in a child.
Hand-Schuller-Christian Disease
