Chapter 6: High yield White Blood Cell Disorders

Question 1

CD 34

Answer 1

hematopoietic stem cell

Question 2

CD 16

Answer 2

Fc receptor. Decreased in immature neutrophils.

Question 3

Left shift?

Answer 3

release immature neutrophils

Question 4

Eosinophilia

Answer 4

Think Hodgkin Lymphoma.

Question 5

Basophilia

Answer 5

Think chronic myeloid leukemia

Question 6

Positive TdT nuclear staining means

Answer 6

Its a lymphoblast - so would be ALL. TdT is a DNA polymerase

Question 7

Tdt+; CD10,19, and 20. t(12;21) [children] t(9;22) [adults]

Answer 7

B-ALL

Question 8

TdT+; CD2-CD8 NO CD10

Answer 8

T-ALL. if present in teens as a mediastinal (thymic) mass - call acute lymphoblastic LYMPHOMA because the malignant cells form a mass.

Question 9

MPO positive in cytoplasm means

Answer 9

Myeloblasts. will get crystal aggregates of MPO into AUER RODS

Question 10

MPO +; t(15;17)

Answer 10

APL (Acute promyelocytic leukemia). retinoic acid receptor (17). Risk of DIC. Tx = all-trans-retinoic acid (vitamin A derivative)

Question 11

Cytopenia; hypercellular bone marrow; increased blasts (but < 20%); prior exposure to alkylating agents or radiotherapy

Answer 11

Myelodysplastic syndrome

Question 12

Neoplastic proliferation of blasts; defiend as the accumation of >20% blasts in the bone marrow

Answer 12

Acute Leukemia

Question 13

Neoplastic proliferation of mature circulating lymphocytes; characterized by a high WBC count

Answer 13

Chronic Leukemia

Question 14

Neoplastic proliferation of naïve B cells that co-express CD5 and CD20. Increased lymphocytes and smudge cells. Generalized lymphadenopathy.

Answer 14

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

Question 15

Neoplastic proliferation of mature B cell characterized by HAIRY CYTOPLASMIC PROCESSES. + tartrate-resistant acid phosphatase (TRAP). splenomegaly [RED PULP], dry tap on bone marrow

Answer 15

Hairy Cell leukemia

Question 16

Tx of hairy cell leukemia

Answer 16

2-CDA (Cladribine) - an adenosine deaminase inhibitor

Question 17

Neoplastic proliferation of mature CD4+ T cells. Associated with HTLV-1. Rash, generalized lymphadenopathy; hepatosplenomegaly; lytic bone lesions with hypercalcemia

Answer 17

Adult T-Cell leukemia/lymphoma (ATLL)

Question 18

Neoplastic proliferation of mature CD4+ T cells. Pautrier microabscesses. Sezary Syndrome

Answer 18

Mycosis Fungoides

Question 19

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.

Answer 19

Chronic Myeloid Leukemia (CML). Can transform to AML (2/3) or ALL (1/3).

Question 20

Tx of CML

Answer 20

Imatinib - blocks tyrosine kinase activity

Question 21

Neoplastic proliferation of mature myeloid cells, especially RBC. Associated with JAK2 kinase mutation. Itching after bathing. EPO is decreased.

Answer 21

Polycythemia Vera. tx: phlebotomy. Second line = hydroxyurea

Question 22

Neoplastic proliferation of mature myeloid cells, especially platelets. Associated with JAK2 kinase mutation.

Answer 22

Essential Thrombocythemia (ET)

Question 23

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

Answer 23

Myelofibrosis

Question 24

Rheumatoid arthritis and early stages of HIV infection show what area of lymph node hyperplasia

Answer 24

Follicular hyperplasia (B-cell region)

Question 25

What region of the lymph node undergoes hyperplasia with viral infections (infectious mononucleosis)

Answer 25

Paracortex hyperplasia (T-cell region)

Question 26

When do you see hyperplasia of sinus histiocytes in lymph nodes

Answer 26

nodes that are training tissue with cancer

Question 27

Neoplastic proliferation of lymphoid cells that forms a mass. May arise in a lymph node or in extranodal tissue

Answer 27

Lymphoma

Question 28

Neoplastic proliferation of small B cells (CD20). Late adult. painless lymphadenopathy. t(14:18)

Answer 28

Follicular Lymphoma. BCL2 on Chromosome 18. CAN PROGRESS TO DIFFUSE LARGE B-CELL LYMPHOMA - presents as an enlarging lymph node

Question 29

Tx of Follicular lymphoma.

Answer 29

symptomatic only. Low-dose chemo or rituximab (anti-CD20 antibody)

Question 30

Neoplastic proliferation of small B cells (CD20). T(11;14). Late adult, painless lymphadenopathy.

Answer 30

Mantel Cell lymphoma. Cyclin D1 gene on chromosome 11 Translocates to Ig heavy chain. Cyclin D1 promotes G1/S transition.

Question 31

Tx of Mantel Cell lymphoma

Answer 31

Ibrutinib

Question 32

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

Answer 32

Burkitt Lymphoma

Question 33

Neoplastic proliferation of large B cells (CD20) that grow diffusely in sheets. Late adult hood as an enlarging lymph node or extranodal mass.

Answer 33

Diffuse Large B-cell lymphoma. tx: R-CHOP

Question 34

Neoplastic proliferation of Reed-Sternberg cells (large B cell, multi lobed 'owl eye'). CD15, CD30. Spread = contiguous

Answer 34

Hodgkin Lymphoma.

Question 35

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.

Answer 35

Multiple Myeloma. | Neoplastic plasma cells activate the RANK receptor on osteoclast. see in vertebrae and skull.

Question 36

CRAB Criteria for Multiple Myeloma

Answer 36

Calcium, Renal dysfunction, Anemia, Bone

Question 37

M Spike <3g/Dl; no CRAB criteria;

Answer 37

Monoclonal Gammopathy of Undetermined Significance

Question 38

B-cell Lymphoma with monoclonal IgM production. Mspike; Visual and neurological deficits; Bleeding. No lytic bone lesions.

Answer 38

Waldenstrom Macroglobulinemia Acute complications are treated with plasmapheresis

Question 39

Birbeck (Tennis Racket) granules; CD1a; S100+

Answer 39

Langerhans cell histiocytosis

Question 40

Malignant proliferation of Langerhans cells. Skin rash and cystic skeletal defects in an infant. Rapidly fatal.

Answer 40

Letterer-Siwe Disease

Question 41

Benign proliferation of Langerhans cells in bone. Pathologic fracture in adolescent. Skin not involved.

Answer 41

Eosinophilic Granuloma

Question 42

Malignant proliferation of Langerhans cells. Scalp rash; lytic skull defects; diabetes insipidus; exophthalmos in a child.

Answer 42

Hand-Schuller-Christian Disease