05 Lymphocytosis and Lymphoid leukemias

Question 0

B-ALL

Answer 0

Bcell acute lymphoblastic leukemia
Primarily children
*Pos for CD34+, CD19+, CD10+, and TdT+
CD20 -

Good prognosis: Hyperploidy and t(12:21) (most common in kidos)
Bad prognosis: Hypoploidy, t(9;22) “philadelphia chromosome”, 11q23 (MLL abnormalities), 10yo, high WBC, CSF involvement, marrow involvement post treatment.

New treatments with anti tumor chimeric T cells

Question 1

Absolute lymphocytosis

Answer 1

WBC * Lymphocytes
>4k is lymphocytosis

Neoplastic Process likely if:
Chronic
Homogenous
20-40k Absolute lymphocyte (reactive can go 10-12)
Significant weightloss and fatigue

Question 2

T-ALL

Answer 2

Adolescent males

Tcell acute lymphoblastic leukemia
POS for CD1a+, CD3+, CD4+, CD8+, TdT

Thymic and mediastinal mass

POOR PROGNOSIS

Question 3

CLL

Answer 3

Chronic Lymphocytic Leukemia - Bcell
Indolent in older adults - may have autoimmune thrombocytopenias, ANEMIAS FROM BM REPLACEMEN
Soccerrball or gingersnap chromatin

POS for CD5+, CD19+, CD20+, CD23+

Good prog: 13q, mutated IgHV (mature)
Intermediate: Trisomy 12
Bad prog: 11q deletion, 17pdeletion(p53), unmutated IgHV(naiive)

Rituximab treatment!

Question 4

Hairy Cell Leukemia

Answer 4

Indolent in 40-60 yo males with pancytopenia and splenomegaly (red pulp infiltration)

Associated with marrow fibrosis

POS for CD19+, CD20+, CD11c, CD22
TRAP+

Question 5

Sezary Syndome/Mycoses fungoids

Answer 5

CD4+ Tcell neoplasm

Erythroderma, lymphadenopathy, Sezary cells with cerebriform nuclei

Question 6

LGLL

Answer 6

Tcell large granular lymphocytic leukemia
CD8+ Tcells or NK
Lymphocytes with pink granules

• Indolent disorder chracterized by neutropenia, anemia, and splenomegaly
• Associated with Rheumatoid arthritis

Question 7

ATLL

Answer 7

Adult Tcell leukemia/ymphoma
CD4+, CD25+
HTLV1 provirus- travel to japan, west africa, carribean
Hypercalcemia

“Flower cells”
POOR PROGNOSIS, AGRESSIVE

Question 8

How do we check for clonality in B cells?

Answer 8

• Light chain restriction
  
  • Kappa vs. Lambda
• IgH variable genePCR
• Imunophenotyping for aberrancy

Question 9

T/NK cell clonality assessment

Answer 9

• Tcell receptorr PCR

* Immunocytochemistry for aberrancy

Question 10

Acute leukemia

Answer 10

>20% blasts in blood or BM
CAn involve lymph tissue
AML vs ALL
   -Myeloid
       * Auer rods, CD13,14, 15, 33, 117
   -Lymphoid
       * CD3, 19, 20

Question 11

Dasatinib

Answer 11

Tyrosine kinase inhibitor for t(9;22) “philadelphia chromosome” positive ALL

Question 12

CLL treatment indications

Answer 12

Rapid lymphocyte doubling time
HGB<100k/uL
Autoimmune anemia/thromboctopenia
Large cell transformation
Enlarging symptomatic lymph nodes

Treat with BTK inhibitor and Rituximab