White Blood Cell Disorders

Question 1

Generally speaking, how can you distinguish NHL from HL?

Answer 1

NHL appears with HS that involves the entire node and that has cells that are all malignant, while HL will present with Reed Stemberg cells surrounded by tons of lymphocytes.

Question 2

What CD are NHL B-cells positive For?

Answer 2

CD20

Question 3

What are the low grade NHL?

Answer 3

CLL/SLL, Mantle cell (t 11,14), Follicular (t 14,18), Marginal (t 11,18), and Lymphoplasmacytoid (t 9,14)

Question 4

What are the high grade NHL?

Answer 4

Diffuse large B cell (t 14,18), and Burkitt’s (t 8,14), (t 2,8), or (t 8,22)

Question 5

What systems are involved in CLL/SLL?

Answer 5

Expect involvement of bone marrow and lymph node. Associated with trisomy 12.

Question 6

What HS finding for CLL/SLL?

Answer 6

Presence of smudge cells.

Question 7

What occurs in Mantle cell lymphoma?

Answer 7

Overproduction of Cyclin D1 (t 11 14)

Question 8

What occurs in Follicular cell lymphoma?

Answer 8

Overproduction of Bcl2 (t 14 18). It is more pale nodular. Associate with markers CD19, CD20, and CD10

Question 9

What occurs in Marginal cell lymphoma?

Answer 9

Associated with Trisomy 18 (t 11,18). History would include h. pylori, hashimoto’s, or sjogrens.

Question 10

What occurs in lymphoplasmacytoid lymphoma?

Answer 10

Cells are produced that are half lymphocyte/half plasma cell (t 9 14). Associated with Waldenstrom’s Macroglobulinemia.

Question 11

What makes a lymphoma high grade?

Answer 11

It proliferates rapidly.

Question 12

What translocation is diffuse B cell lymphoma? What CD markers does it show?

Answer 12

t 14 18

Arises usually from follicular NHL.

CD19, CD20, CD22

Question 13

What occurs in Burkitt’s lymphoma? What do you expect on HS? What translocations is it confirmed with?

Answer 13

C-myc mutation associated usually with EBV

Starry sky appearance on scope

t 8 22, t 8 14, t 2 8

Question 14

What CD marker is associated with the cells in HL?

Answer 14

CD15/30

Question 15

What two types of HL are there?

Answer 15

Classical (Mixed cellularity, nodular sclerosis, lymphocyte depletion, and lymphocyte rich classic HL)
Nodular Lymphocyte Predominant HL

Question 16

What cells will nodular lymphocyte predominant HL present with?

Answer 16

Lacunar cells surrounded by fibrosis/sclerosis

Question 17

What clinical symptoms are associated with HL?

Answer 17

Low grade fever, night sweats, weight loss, mediastinal lymphadenopatjy

Question 18

What are the four plasma cell disorders?

Answer 18

Multiple myeloma, Monoclonal Gammopathy of Undetermined Significance, Waldenstrom Macroglobulinemia, and Langerhan Cell Histiocytes

Question 19

Multiple myeloma is the malignant proliferation of plasma cells that typically occurs in older men. What clinical features do you expect?

Answer 19

Bone lesions with hypercalcemia, Rouxleaus formation of RBCs, and primary AL amylooidosis (kappa light chains).

Expect an M spike, but do not confuse with MGUS.

Question 20

What does MGUS show on SPEP?

Answer 20

It shows an increased M spike. You will know it from MM, because it will have none of the MM clinical manifestations.

Question 21

Waldenstrom Macroglobulinemia is a B-cell lymphoma with monoclonal IgM production associated with lymphoplasmacytoid NHL. What clinical features do you expect?

Answer 21

Neurological defects with an M spike on SPEP.

Question 22

What is the main granule that shows for Langerhan Cell Histiocytes?

Answer 22

Birbeck granules!

Question 23

How can you tell a vignette is an eosinophilic granuloma (Langerhan’s Cell Histiocyte?)

Answer 23

It will be a single bony lesion, with S100+ and CD19+ markers.

Question 24

How can you tell a vignette is an Hand-Schuler-Christian (Langerhan’s Cell Histiocyte?)

Answer 24

Multiple lesions will be present, with diabetes insipidus likely happening in the child.

Question 25

How can you tell a vignette is an Letterer-Siwe (Langerhan's Cell Histiocyte?)

Answer 25

It will be an infant with a skin rash who has multiple lesions and poor prognosis.

Question 26

How can you distinguish myelodysplastic, myeloproliferative, and leukemia conditions?

Answer 26

Myelodysplastic is abnormal maturation of cell lines, myeloproliferative is overproduction of cell lines, and leukemia is overproduction of ONE type of cell.

Question 27

Myeloproliferative disorders include CML, PV, ET, and myelofibrosis. What cell line is most prominant in CML? What is its characteristic issue? How do you treat it?

Answer 27

CML = basophils Driven by Philly chromosome (t 9 22): Bcr-abl Treatment is GLEEVNEC/imatinib

Question 28

Myeloproliferative disorders include CML, PV, ET, and myelofibrosis. What cell line is most prominant in PV? What is its characteristic issue? How do you treat it?

Answer 28

PV = RBCs Associated with JAK2 kinase mutation Complete a phlebotomy for viscous blood, then hydroxyurea. Expect an increased RBC volume, normal arterial oxygen saturation, and splenomegaly with leukocyte ALP >100.

Question 29

Myeloproliferative disorders include CML, PV, ET, and myelofibrosis. What cell line is most prominant in ET? What is its characteristic issue? How do you treat it?

Answer 29

ET = platelets JAK 2 kinase mutation Treat with hydroxyurea and low-dose aspirin

Question 30

Myeloproliferative disorders include CML, PV, ET, and myelofibrosis. What cell line is most prominant in myelofibrosis? What is its characteristic issue? How do you treat it?

Answer 30

Myelofibrosis = megakaryocytes Associated with JAK 2 mutation Megakaryocytes produce excess PDGF causing marrow fibrosis.

Question 31

You should consider a myelodysplastic disorder if a patient presents with an anemia that has no other explanation. What are the three subtypes? What characteristic finding on HS?

Answer 31

Ringed sideroblasts 1. Refractory anemia with excess blasts (RA-EB) 2. Refractory anemia with excess blasts in transformation 3. Chronic myelomonocytic leukemia

Question 32

Acute leukemia is characterized by "blasts," which are immature WBCs with "punched out" nucleoli. It has two types: ALL and AML. What is ALL?

Answer 32

Acute lymphoblastic leukemia

Question 33

What is characteristic of ALL?

Answer 33

Presence of TdT, a mediastinal mass in a child is probably T-cell ALL

Question 34

What types of ALL are there?

Answer 34

B-cell (more common, but plague adults leading to poor prognosis) T-cell (more common in kids and has good prognosis)

Question 35

Acute leukemia is characterized by "blasts," which are immature WBCs with "punched out" nucleoli. It is also associated with Auer rods and granules in cytoplasm. It has two types: ALL and AML. What is AML?

Answer 35

Acute myeloid leukemia

Question 36

What population is AML most common?

Answer 36

Older adults

Question 37

What is characteristic of a type of AML, APL?

Answer 37

Auer rods on HS with a t 15 17 translocation of retinoic acid receptor. Treat with all trans retinoic acid.

Question 38

What is characteristic of a type of AML, acute monocytic leukemia?

Answer 38

Proliferation of monoblasts, usually lack TPO, and blasts are characteristically infiltrating the gums

Question 39

What is characteristic of a type of AML, acute megakaryoblastic leukemia?

Answer 39

Associated with Down syndrome.

Question 40

From what condition can AML arise?

Answer 40

Myelodysplastic disorders (i.e. RA-EB, etc)

Question 41

Chronic leukemias include CLL, hairy cell leukemia, and adult T cell leukemia/lymphoma. What is characteristic of CLL?

Answer 41

CLL involves proliferation of naive B cells that co-express CD5 and CD20. It is the most common leukemia overall. Will see "smudge" cells on HS Treat with rituxamab. Often leads to SLL

Question 42

Chronic leukemias include CLL, hairy cell leukemia, and adult T cell leukemia/lymphoma. What is characteristic of hairy cell leukemia?

Answer 42

Occurs in middle aged men usually. Involves proliferation of mature B cells characterized by hairy cell processes. Cells are TRAP positive. Usually "dry tap" on aspirate Treat with 2-CDA cladribine

Question 43

Chronic leukemias include CLL, hairy cell leukemia, and adult T cell leukemia/lymphoma. What is characteristic of adult T cell leukemia/lymphoma?

Answer 43

Proliferation of CD4+ T cells. Associated with HTLV-1 infection.