HAEM5:T-prolymphocytic leukaemia: Difference between revisions
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T-PLL is a rare disorder, comprising about 2% of all mature lymphoid leukemia cases in adults. It primarily occurs in the elderly, with a median age of 65 years (ranging from 30 to 94 years) and shows a slight male predominance with a male to female ratio of 1.33:1. | T-PLL is a rare disorder, comprising about 2% of all mature lymphoid leukemia cases in adults. It primarily occurs in the elderly, with a median age of 65 years (ranging from 30 to 94 years) and shows a slight male predominance with a male to female ratio of 1.33:1. | ||
==Clinical Features== | ==Clinical Features== | ||
The most prevalent symptom of the disease is a leukemic presentation, characterized by a rapid, exponential increase in lymphocyte counts, which exceed 100 × 10^9/L in 75% of patients. Approximately 30% of patients may initially experience an asymptomatic, slow-progressing phase, but this typically develops into an active disease state | The most prevalent symptom of the disease is a leukemic presentation, characterized by a rapid, exponential increase in lymphocyte counts, which exceed 100 × 10^9/L in 75% of patients. Approximately 30% of patients may initially experience an asymptomatic, slow-progressing phase, but this typically develops into an active disease state. | ||
{| class="wikitable" | {| class="wikitable" | ||
|'''Signs and Symptoms''' | |'''Signs and Symptoms''' | ||
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Hepatosplenomegaly (Frequently observed) | Hepatosplenomegaly (Frequently observed) | ||
Generalized lymphadenopathy with slightly enlarged lymph nodes (Frequently observed | Generalized lymphadenopathy with slightly enlarged lymph nodes (Frequently observed) | ||
Cutaneous involvement (20%) | Cutaneous involvement (20%) | ||
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Atypical lymphocytosis > 5 × 10^9/L | Atypical lymphocytosis > 5 × 10^9/L | ||
Serum lactate dehydrogenase (LDH) (increased) | Serum lactate dehydrogenase (LDH) (increased-may reflect disease burden) | ||
β 2 microglobulin (B2M) (increased-may reflect disease burden) | |||
|} | |} | ||
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|Loss | |Loss | ||
|14q | |14q | ||
| | |14q32.1 | ||
| | |Yes | ||
| | |Yes | ||
| | |No | ||
| | |Major diagnostic criteria | ||
|- | |- | ||
|17 | |17 | ||
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|Loss | |Loss | ||
|Monosomy 22 | |Monosomy 22 | ||
22q | del(22q) | ||
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