Other Lymphomas and Related Neoplasms

Question 1

What is the difference between B-ALL and B-LBL? Which is the most common form of childhood leukemia?

Answer 1

ALL is acute lymphoblastic leukemia, mostly in bone marrow and peripheral blood
LBL is lymphoblastic lymphoma, mostly in extramedullary tissues like lymph nodes
B-ALL is the most common childhood leukemia

Question 2

Precursor B-cell ALL and CML can both involve t(9;22), but which one has a 190-kd product and which has a 210-kd?

Answer 2

CML is 210-kd; B-ALL is 190-kd

Question 3

Compared to B-ALL, where is T-ALL likely to present?

Answer 3

More likely to be extramedullary and lymphomatous (esp. the mediastinal lymph nodes in adolescent males)

Question 4

Compared to mature B-cell lymphomas, how common and what prognosis is associated with T-cell and NK-cell lymphomas?

Answer 4

T-cell and NK-cell lymphomas are less common and have a poorer prognosis (usually higher stage and more aggressive at presentation)

Question 5

Where do precursor vs. mature T-cell neoplasms arise?

Answer 5

Precursor - in the thymus; mature - outside the thymus (ex. lymph nodes, spleen, GI tract, skin)

Question 6

What neoplasm is connected to HTLV-1 endemic areas, such as SW Japan?

Answer 6

ATLL (adult T-cell leukemia/lymphoma)

Question 7

Name the most common primary cutaneous T-cell lymphoma and its 3 stages.

Answer 7

Mycosis fungoides; eczematous stage - plaque stage - tumor stage

Question 8

Which stage of mycosis fungoides involves mycosis cells: large lymhpoid cells with hyperchromatic, cerebriform nuclei?

Answer 8

Plaque stage

Question 9

What syndrome associated with mycosis fungoides is characterized by erythroderma, generalized lymphadenopathy, and circulating lymphoma cells in the peripheral blood?

Answer 9

Sezary syndrome

Question 10

What mature T-cell neoplasm is associated with t(2;5) ALK-NPM and CD30+ hallmark cells?

Answer 10

Anaplastic large cell lymphoma

Question 11

Aggressive, systemic mature T-cell neoplasm associated with EBV, skin rash, cold agglutinins, hemolytic anemia, circulating immune complexes and positive rheumatoid factor?

Answer 11

Angioimmunoblastic T-cell lymphoma

Question 12

Most common malignancy of Americans 10-30 years old, derived from germinal center B-cells and presents as nontender peripheral adenopathy of a single node or group of nodes?

Answer 12

Hodgkin lymphoma

Question 13

Which type of Hodgkin lymphoma is characterized by Reed-Sternberg cells? by lymphocytic and histiocytic “popcorn” cells?

Answer 13

Classical HL; nodular lymphocyte-predominant HL

Question 14

Most common subtype of classical Hodgkin lymphoma, characterized by fibrous thickening of the lymph node capsule and mediastinal involvement?

Answer 14

Nodular sclerosis Hodgkin lymphoma

Question 15

Burkitt lymphoma, classical Hodgkin lymphoma, and post-transplant lymphoproliferative disorders have all been associated with what virus?

Answer 15

EBV

Question 16

Which kind of PTLD (post-transplant lymphoproliferative disorder) is characterized by clonal Igs and commonly progresses to diffuse large B-cell lymphoma?

Answer 16

Monomorphic PTLD

Question 17

Iatrogenic immunodeficiency-associated lymphoproliferative disorders are usually seen in patients with what 2 criteria?

Answer 17

Taking immunosuppressive drugs and EBV positive

Question 18

What class of disorders is caused by dysregulated cytokines and CD8 T cells, leading to macrophages that engulf normal hematopoietic cells in the bone marrow, spleen, and lymph nodes?

Answer 18

Hemophagocytic disorders

Question 19

Histiocytic neoplasms are rare disorders that can arise from what 3 kinds of cells?

Answer 19

Macrophages, dendritic cells, and Langerhans cells

Question 20

What neoplasm arises from a proliferation of interdigitating reticulum cells, and is positive for S-100, CD1a, and Birbeck granules?

Answer 20

Langerhans cell histiocytosis

Question 21

What is the least aggressive form of Langerhans cell histiocytosis, which presents as a self-limited bone disorder to older persons?

Answer 21

Eosinophilic granuloma

Question 22

What form of Langerhans cell histiocytosis is a multifocal, indolent disorder traditionally characterized by diabetes insipidus, proptosis/exopthalmus, and defects in membranous bones?

Answer 22

Hand-Schuller-Christian disease

Question 23

What form of Langerhans cell histiocytosis is a rare, acute, disseminated variant found in kids

Answer 23

Letterer-Siwe disease

Question 24

Which part of the spleen (white pulp or red pulp) consists of B cells and T cells ensheathing a central artery?

Answer 24

White pulp (the red pulp = stromal cords and vascular sinuses)

Question 25

Which part of the spleen (white pulp or red pulp) is primarily a filter that screens and eliminates defective or foregin cells, including RBC inclusions?

Answer 25

Red pulp

Question 26

What hematopoietic disease is characterized by hypersplenism and Gamna-Gandy bodies (foci of old hemorrhages turned into fibrotic nodules) followed by acquired asplenia?

Answer 26

Sickle cell anemia

Question 27

What is the most common prirmary malignant tumor of the spleen?

Answer 27

Hemangiosarcoma

Question 28

Severe combined immunodeficiency, DiGeorge syndrome, Wiskott-Aldrich syndrome, Swiss-type hypogammaglobulinemia, and ataxia telangiectasia are all associated with defects in what organ of the body?

Answer 28

Thymus

Question 29

What disease is most commonly associated with thymic hyperplasia and thymoma?

Answer 29

Myasthenia gravis