Sandbox: T-cell prolymphocytic leukemia: Difference between revisions
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*Common complications of t-cell prolymphocytic leukemia, include: | *Common complications of t-cell prolymphocytic leukemia, include: | ||
*Prognosis is generally poor, and the 5 year survival rate of patients with t-cell prolymphocytic leukemia is approximately | *Prognosis is generally poor, and the 5 year survival rate of patients with t-cell prolymphocytic leukemia is approximately | ||
== Diagnosis == | == Diagnosis == | ||
=== Symptoms === | === Symptoms === | ||
Revision as of 22:26, 23 May 2016
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [2]
Synonyms and keywords: T-cell chronic lymphocytic leukemia, "Knobby" type of T-cell leukemia, T-prolymphocytic leukemia/T-cell lymphocytic leukemia- Kiel, T-PLL
Overview
T-cell-prolymphocytic leukemia (also known as T-PLL) is a mature T-cell leukemia with aggressive behavior and predilection for blood, bone marrow, lymph nodes, liver, spleen, and skin involvement.[1]
Historical Perspective
- T-cell prolymphocytic leukemia was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event].
Classification
- T-cell prolymphocytic leukemia may be classified according to World Health Organization (WHO) classification system into subtypes:
- [group1]
- [group2]
- [group3]
Pathophysiology
- The pathogenesis of t-cell prolymphocytic leukemia is characterized by:
- The has been associated with the development of t-cell prolymphocytic leukemia.
- On gross pathology, characteristic findings of t-cell prolymphocytic leukemia, include:
- On microscopic histopathological analysis, characteristic findings of t-cell prolymphocytic leukemia, include:
Causes
- Common causes of T-cell prolymphocytic leukemia, include:
Differentiating T-cell Prolymphocytic Leukemia from Other Diseases
- T-cell prolymphocytic leukemia must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as:
- [Differential dx1]
- [Differential dx2]
- [Differential dx3]
Epidemiology and Demographics
- The prevalence of t-cell prolymphocytic leukemia is approximately [number or range] per 100,000 individuals worldwide.
- T-cell prolymphocytic leukemia is very rare, and it represents 2% of all small lymphocytic leukemias in adults.
Age
- T-cell prolymphocytic leukemia is more commonly observed among patients aged between 30 to 40 years old.
- T-cell prolymphocytic leukemia is more commonly observed among young adults.
Gender
- T-cell prolymphocytic leukemia affects men and women equally.
Race
- There is no racial predilection for t-cell prolymphocytic leukemia.
Risk Factors
- Common risk factors in the development of t-cell prolymphocytic leukemia are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].
Natural History, Complications and Prognosis
- The majority of patients with t-cell prolymphocytic leukemia remain asymptomatic for [duration/years].
- Early clinical features, include
- If left untreated, patients with t-cell prolymphocytic leukemia may progress to develop
- Common complications of t-cell prolymphocytic leukemia, include:
- Prognosis is generally poor, and the 5 year survival rate of patients with t-cell prolymphocytic leukemia is approximately
Diagnosis
Symptoms
- Symptoms of t-cell prolymphocytic leukemia may include the following:
- Fever
- Weight loss
- Night sweats
Physical Examination
- Patients with t-cell prolymphocytic leukemia usually appear pale and malnourished.
- Physical examination may be remarkable for:
- Hepatosplenomegaly
- Generalized lymphadenopathy
- Skin infiltration
Peripheral Blood Smear
- Medium-sized lymphocytes
- Single nucleoli and basophilic cytoplasm
- The nuclei are usually round to oval in shape,
- Irregular nuclear outline that is similar to the cerebriform nuclear shape seen in Sézary syndrome.
- A small cell variant comprises 20% of all T-PLL cases, and the Sézary cell-like (cerebriform) variant is seen in 5% of cases
Laboratory Findings
- Laboratory findings consistent with the diagnosis of t-cell prolymphocytic leukemia, include:
- High lymphocyte count (> 100 x 109/L)
- Anemia
- Thrombocytopenia
Imaging Findings
- There are no specific imaging findings associated with t-cell prolymphocytic leukemia.
Treatment
Medical Therapy
- There is no treatment for t-cell prolymphocytic leukemia; the mainstay of therapy is supportive care.
- The mainstay of therapy for t-cell prolymphocytic leukemia is [medical therapy 1] and [medical therapy 2].
- [Medical therapy 1] acts by [mechanism of action1].
- Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].
Surgery
- Surgery is the mainstay of therapy for t-cell prolymphocytic leukemia.
- [Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of t-cell prolymphocytic leukemia.
- [Surgical procedure] can only be performed for patients with [disease stage] t-cell prolymphocytic leukemia.
Prevention
- There are no primary preventive measures available for t-cell prolymphocytic leukemia.
References
- ↑ Jaffe E.S., Harris N.L., Stein H., Vardiman J.W. (eds): World Health Organization Classification of Tumors. Pathology and Genetics of Tumours of Haemopoietic and Lymphoid Tissues. IARC Press: Lyon 2001