Sandbox: T-cell large granular lymphocyte leukemia
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [2] Associate Editor(s)-in-Chief: Raviteja Guddeti, M.B.B.S. [3] Maria Fernanda Villarreal, M.D. [4]
Synonyms and keywords: LGL leukemia; Tγ-lymphoproliferative disorder; T-cell chronic lymphocytic leukemia; proliferation of large granular lymphocytes (LGLs)
Overview
T-cell large granular lymphocyte leukemia ( also known as T-GLL) is a rare type of leukemia that exhibits a unexplained, chronic (> 6 months) elevation in large granular lymphocytes (LGLs) in the peripheral blood.[1]
Historical Perspective
- T-cell large granular lymphocyte leukemia was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event].
Classification
- T-cell large granular lymphocyte leukemia may be classified into 2 groups:
- T-cell large granular lymphocyte leukemia (T-LGL)
- Natural-killer (NK) granular lymphocyte leukemia (NK-LGL)
- Other variants of T-cell large granular lymphocyte leukemia include
Pathophysiology
- The pathogenesis of T-cell large granular lymphocyte leukemia is characterized by involvement of cytotoxic-T cells.
- The [gene name] gene/Mutation in [gene name] has been associated with the development of T-cell large granular lymphocyte leukemia, involving the [molecular pathway] pathway.
- On gross pathology, characteristic findings of T-cell large granular lymphocyte leukemia, include:
- T-LGL can be found in peripheral blood, bone marrow, spleen, and liver
- On microscopic histopathological analysis, characteristic findings of T-cell large granular lymphocyte leukemia, include:
- Neoplastic lymphocytes (large in size)
- Presence of azurophilic granules (contains proteins involved in cell lysis such as perforin and granzyme B)
- Bone marrow involvement in this disease is often present, but to a variable extent
- The lymphocytic infiltrate is usually interstitial, but a nodular pattern rarely occurs
Causes
- Common causes of T-cell large granular lymphocyte leukemia, include:
Differentiating T-cell Large Granular Lymphocyte Leukemia from Other Diseases
- T-cell large granular lymphocyte leukemia must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as:
Epidemiology and Demographics
- T-cell large granular lymphocyte leukemia is a rare form of leukemia, comprising 2-3% of all cases of chronic lymphoproliferative disorders.
Age
- Patients of all age groups may develop T-cell large granular lymphocyte leukemia.
- T-cell large granular lymphocyte leukemia is more commonly observed among patients aged [age range] years old.
- T-cell large granular lymphocyte leukemia is more commonly observed among [elderly patients/young patients/children].
Gender
- T-cell large granular lymphocyte leukemia affects men and women equally.
Race
- There is no racial predilection for T-cell large granular lymphocyte leukemia.
Risk Factors
- Common risk factors in the development of T-cell large granular lymphocyte leukemia, include:
Natural History, Complications and Prognosis
- The majority of patients with T-cell large granular lymphocyte leukemia may be initially asymptomatic.
- Early clinical features include
- If left untreated, patients with T-cell large granular lymphocyte leukemia may progress to develop infections.
- Common complications of T-cell large granular lymphocyte leukemia, include:
- Prognosis is generally poor, and the 5 year survival rate of patients with T-cell large granular lymphocyte leukemia is approximately [#%].
Diagnosis
Diagnostic Criteria
- The diagnosis of T-cell large granular lymphocyte leukemia is made when at least [number] of the following [number] diagnostic criteria are met:
- Clonal rearrangements of the T-cell receptor (TCR) genes
- [criterion 2]
- [criterion 3]
- [criterion 4]
Symptoms
- T-cell large granular lymphocyte leukemia is usually asymptomatic.
- Symptoms of T-cell large granular lymphocyte leukemia may include the following:
- [symptom 1]
- [symptom 2]
- [symptom 3]
- [symptom 4]
- [symptom 5]
- [symptom 6]
Physical Examination
- Patients with T-cell large granular lymphocyte leukemia usually appear pale and malnourished.
- Physical examination may be remarkable for:
- [finding 1]
- [finding 2]
- [finding 3]
- [finding 4]
- [finding 5]
- [finding 6]
Laboratory Findings
- There are no specific laboratory findings associated with T-cell large granular lymphocyte leukemia.
- A [positive/negative] [test name] is diagnostic of T-cell large granular lymphocyte leukemia.
- An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of T-cell large granular lymphocyte leukemia.
- Other laboratory findings consistent with the diagnosis of T-cell large granular lymphocyte leukemia include [abnormal test 1], [abnormal test 2], and [abnormal test 3].
Imaging Findings
- There are no [imaging study] findings associated with T-cell large granular lymphocyte leukemia.
- [Imaging study 1] is the imaging modality of choice for T-cell large granular lymphocyte leukemia.
- On [imaging study 1], T-cell large granular lymphocyte leukemia is characterized by [finding 1], [finding 2], and [finding 3].
- [Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].
Other Diagnostic Studies
- T-cell large granular lymphocyte leukemia may also be diagnosed using [diagnostic study name].
- Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].
Treatment
Medical Therapy
- There is no treatment for T-cell large granular lymphocyte leukemia; the mainstay of therapy is supportive care.
- The mainstay of therapy for T-cell large granular lymphocyte 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 large granular lymphocyte leukemia.
- [Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of T-cell large granular lymphocyte leukemia.
- [Surgical procedure] can only be performed for patients with [disease stage] T-cell large granular lymphocyte leukemia.
Prevention
- There are no primary preventive measures available for T-cell large granular lymphocyte leukemia.
*Once diagnosed and successfully treated, patients with T-cell large granular lymphocyte leukemia are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].