Bangalore Call Girl Whatsapp Number 100% Complete Your Sexual Needs
T-PLL.pptx
1. A rare cause of lymphocytosis
Dr. Soma Pradhan
Senior Resident
Department of Hematology
Sir Ganga Ram Hospital, New Delhi.
2. HISTORY
On 13th July 2019, a 42 yrs old male presented to
Clinical Hematology OPD with a complaints of pain
abdomen since last 15 days.
There was no history of fever and weight loss.
On examination, there is no pallor, lymphadenopathy
and spleen was not palpable.
3. The patient was evaluated outside:
CBC:
Hb- 13.5 gm/dl
TLC : 31,200/µl
Platelet count :1,16,000/µl
Peripheral smear examination:
Marked lymphocytosis with 85% lymphocytes and
atypical lymphoid morphology.
20. T-cell Prolymphocytic Leukemia:(T-PLL)
T-PLL accounts for 2% of mature lymphocytic leukemia
in adults.
Median Age : 61 yrs (30 -94 yrs)
Male >>> Female
Rare, aggressive T-cell neoplasm.
Patients with ataxia telangiectasia are at increased risk of
developing T-PLL
21. CLINICAL FEATURES
Lymphocytosis with lymphocyte count >100 x 109/L
Generalised lymphadenopathy
Splenomegaly
Skin infiltration – 20% of the cases
Anemia
Thrombocytopenia
22. DIFFERENTIATING FEATURES
Features T-PLL LGL leukemia Sezary
Syndrome
ATLL
Morphology prolymphocyte Large granular
lymphocyte
cerebriform Flower cell
Phenotype CD4+ CD8- CD8+ CD4- CD4+CD8- CD4+ CD8-
CD4+ CD8+ CD57+ CD3+ CD 25 +
CD7+ CD52+ CD16+/CD94+ CD7- CD26- CD7-
Histology
Spleen Red pulp Red pulp - -
Skin dermal - Dermal and
epidermal
Dermal and
epidermal
HTLV-1 Negative Negative Negative Positive
Clinical
course
Aggressive Indolent Chronic Aggressive-
Acute,
lymphatous
types
23. A B
C D
A- T-PLL
B- LGL Leukemia
C- Sezary Syndrome
D- ATLL
24. MOLECULAR GENETICS
T-cell receptor genes are rearranged and are identical, confirming a
clonal expansion of T-cells.
The most frequently observed group of cytogenetic abnormalities
involve chromosome 14.
inv(14) (q11;q32), t(14;14)(q11;q32)
The above translocations juxtaposes between TRA LOCUS with
TCL1A & TCL1B oncogenes at14q32.1
t(X;14)(q28;q11) :MTCP1 (mature T cell proliferation 1 gene),
which is located on the X chromosome.
Cytogenetic abnormalities involving chromosome 8 are the next
most frequently observed (idic(8p11), t(8;8) and trisomy 8q).
25.
26. Other recurrent abnormalities seen with conventional
techniques include :
loss of 11q23 (ATM inactivation) together with additional
losses (22q, 13q, 6q, 9p, 12p,17p) and gains (22q and 6p).
12p13 deletion occurs in 50% of the cases.
highly recurrent, largely exclusive, gain-of-function
mutations involving IL2RG, JAK1/3, and STAT5B,
which lead to constitutive STAT5 signaling.
27.
28. DISEASE PROGRESSION &
PROGNOSIS
T-PLL is aggressive and often resistant to therapy.
Median survival is 1-2 years.
The best responses have been reported with the monoclonal
antibody alemtuzumab (anti―CD52).
Autologous or allogeneic stem cell transplantation.
High levels of expression of both TCL1 and AKT1 have been
identified as poor prognostic markers.
STAT5B mutations have been documented to have a negative
prognostic impact.
Editor's Notes
Lymphadenopathy, although present in a majority of
patients, is rarely bulky. Anemia and thrombocytopenia are seen in up to one-half
of patients.3 Erythematous or nodular skin rashes involving the trunk or limbs, peripheral
edema, and pleuroperitoneal effusions may be seen in up to one-quarter
of patients with T-PLL