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splenic marginal zone lymphoma
ICD-10 Codes
Description
Splenic Marginal Zone Lymphoma (SMZL) Description
Splenic marginal zone lymphoma (SMZL) is a rare type of non-Hodgkin lymphoma that affects the spleen, bone marrow, and frequently the blood. It is characterized by the growth of abnormal B-cells in the marginal zone of the spleen, which can lead to splenomegaly (enlargement of the spleen) and lymphocytosis (increased white blood cells).
Key Features:
- Rare subtype: SMZL accounts for only a small percentage of all non-Hodgkin lymphomas.
- Spleen involvement: The disease primarily affects the spleen, but can also involve the bone marrow and blood.
- B-cell malignancy: SMZL is a type of B-cell cancer that arises from the marginal zone of the spleen.
- Slow-growing: SMZL is considered an indolent (slow-growing) lymphoma, meaning it progresses slowly over time.
Symptoms:
- Splenomegaly (enlargement of the spleen)
- Lymphocytosis (increased white blood cells)
- Fatigue
- Weight loss
- Abdominal pain or discomfort
Diagnosis:
- Histological examination of the spleen tissue
- Blood tests to assess lymphocyte count and other parameters
- Imaging studies, such as CT scans or MRI, to evaluate spleen size and involvement.
References:
- [3] Splenic marginal zone lymphoma (SMZL) is a rare B-cell malignancy involving the spleen, bone marrow, and frequently the blood.
- [13] Splenic marginal zone lymphoma (SMZL) is a type of marginal zone lymphoma, a cancer made up of B-cells that replace the normal architecture of the white pulp of the spleen.
- [11] Splenic marginal zone lymphoma (SMZL) is an uncommon subtype of non-Hodgkin lymphoma that usually presents with splenomegaly and lymphocytosis and is characterized by a marginal zone growth pattern in the spleen.
Additional Characteristics
- Splenic marginal zone lymphoma (SMZL) is a rare type of non-Hodgkin lymphoma that affects the spleen, bone marrow, and frequently the blood.
- The disease primarily affects the spleen, but can also involve the bone marrow and blood.
- It is considered an indolent (slow-growing) lymphoma, meaning it progresses slowly over time.
Signs and Symptoms
Splenic marginal zone lymphoma (SMZL) can manifest in various ways, depending on the individual case. Here are some common signs and symptoms associated with SMZL:
- Enlarged spleen: This is one of the most common symptoms of SMZL, where the spleen becomes larger than normal due to the accumulation of cancerous cells. [1][2]
- No obvious symptoms: In some cases, people with SMZL may not experience any noticeable symptoms at all, and the disease might only be detected during a routine examination or blood test. [7][8]
- B symptoms: Some individuals with SMZL may exhibit B symptoms, which include:
- Fever
- Night sweats
- Weight loss [4]
- Splenomegaly: This refers to the enlargement of the spleen, which can be detected during a physical examination. [2][5]
- Lymphocytosis: This is an increase in the number of lymphocytes (a type of white blood cell) in the blood or bone marrow. [6]
It's essential to note that not everyone with SMZL will experience all of these symptoms, and some people may have no symptoms at all. The presence and severity of symptoms can vary depending on factors such as the patient's age, overall health, and the specific characteristics of the disease.
References:
[1] Context result 2: Splenic marginal zone lymphoma can grow for years before the first symptoms appear. [2] Context result 3: Splenic – The rarest subtype of marginal zone lymphoma is splenic. It affects the spleen, an organ in the chest that is not normally visible or palpable (can be felt) under the skin. [3] Context result 5: GASTRIC MALT LYMPHOMAS Since gastric MALT lymphoma ... [4] Context result 4: B symptoms [5] Context result 2: Splenic marginal zone lymphoma can grow for years before the first symptoms appear. [6] Context result 3: Splenic – The rarest subtype of marginal zone lymphoma is splenic. It affects the spleen, an organ in the chest that is not normally visible or palpable (can be felt) under the skin. [7] Context result 8: In some cases, people with SMZL may not experience any noticeable symptoms at all, and the disease might only be detected during a routine examination or blood test. [8] Context result 7: No obvious symptoms
Additional Symptoms
- Enlarged spleen
- No obvious symptoms
- Lymphocytosis
- splenomegaly
Diagnostic Tests
Splenic marginal zone lymphoma (SMZL) diagnosis involves a combination of clinical features, laboratory tests, and imaging studies.
Clinical Features
- Splenomegaly (enlarged spleen) is a common presentation of SMZL [5].
- Other symptoms may include fatigue, weight loss, and abdominal discomfort [5].
Laboratory Tests
- Complete Blood Count (CBC): A CBC can help identify abnormalities in blood cell counts, which can be indicative of lymphoma [7].
- Blood tests such as LDH test and Beta-2 microglobulin can also be used to diagnose SMZL [8].
- Bone marrow analysis may be performed to confirm the diagnosis and assess the extent of disease [1].
Imaging Studies
- Whole-body computed tomography (CT) scan is considered the "gold standard" investigation for clinical staging of lymphomas, including SMZL [2].
- Other imaging studies such as ultrasound and MRI scans may also be used to evaluate the spleen and other organs.
Additional Tests
- Flowcytometric immunophenotyping can help identify specific cell markers associated with SMZL [3].
- Chromosome analysis may be performed to detect any chromosomal abnormalities [9].
It's essential to note that a combination of these tests is often used to diagnose SMZL, and the specific tests employed may vary depending on individual patient circumstances.
References:
[1] TS dos Santos (2017) - The diagnosis of SMZL can be by the analysis of pathological cells present in bone marrow with blood and spleen analysis not being essential. Bone marrow ...
[2] E Iannitto (2011) - As the “gold standard” investigation for clinical staging of lymphomas, whole-body computed tomography scan allows determining whether the suspected ...
[3] A Mohanpuria (2017) - The diagnosis of SMZL is arrived at by a combination of diagnostic clues like clinical features, peripheral smear findings, flowcytometric immunophenotyping, ...
[5] Jan 12, 2024 - Splenic marginal zone lymphoma (SMZL) is an uncommon subtype of non-Hodgkin lymphoma that usually presents with splenomegaly ...
[7] Jun 23, 2021 - SMZL diagnosis often includes a complete blood count (CBC), in which the levels of each type of blood cell are measured. Other blood tests can ...
[8] Apr 17, 2023 - What tests do providers use to diagnose marginal zone lymphoma? · CBC. · LDH test. · Beta-2 microglobulin, a tumor marker for blood cancers. · Liver ...
Additional Diagnostic Tests
- Complete Blood Count (CBC)
- Blood tests such as LDH test and Beta-2 microglobulin
- Bone marrow analysis
- Whole-body computed tomography (CT) scan
- Ultrasound and MRI scans
- Flowcytometric immunophenotyping
- Chromosome analysis
Treatment
Treatment Options for Splenic Marginal Zone Lymphoma
Splenic marginal zone lymphoma (SMZL) is a rare subtype of non-Hodgkin lymphoma that affects the spleen. While treatment options may vary depending on individual circumstances, several drug treatments have been shown to be effective in managing SMZL.
- Rituximab: This monoclonal antibody has emerged as a preferred approach for treating SMZL. Studies have demonstrated high response rates with rituximab monotherapy, with one study reporting an overall response rate (ORR) of 92% and complete remission (CR) in 44% of patients [15]. Rituximab is often used as the first-line treatment for patients requiring therapy.
- Chemotherapy: In some cases, chemotherapy may be combined with rituximab to enhance treatment efficacy. However, the addition of chemotherapy has not been shown to further improve outcomes in all patients [3].
- Targeted therapies: Other targeted agents, such as Velcade (bortezomib), have also been explored for treating SMZL. These treatments may offer opportunities for highly pretreated individuals or specific patient categories, potentially reducing the need for repeated application of cytotoxic drugs [12].
Key Considerations
When selecting a treatment approach for SMZL, it is essential to consider individual factors such as disease stage and location, overall health, and any lymphoma-related symptoms. Treatment selection should be tailored to each patient's unique needs.
References:
[3] Lage LA de Pádua Covas, et al. (2020). In the last ten years, several studies have shown superiority of rituximab therapy, either alone or in combination [12,13,14], but, in resource-...
[12] New drugs and targeted agents offer treatment opportunities in highly pretreated individuals and in particular categories of patients, often limiting the need for repeated application of cytotoxic drugs. ...
[15] Treatment of splenic marginal zone lymphoma with rituximab monotherapy: progress report and comparison with splenectomy. Oncologist. 2013 ...
Recommended Medications
- Chemotherapy
- Rituximab
- Targeted therapies
💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.
Differential Diagnosis
Differential Diagnosis of Splenic Marginal Zone Lymphoma (SMZL)
Splenic marginal zone lymphoma (SMZL) is a rare subtype of non-Hodgkin lymphoma that typically presents with splenomegaly and lymphocytosis. The differential diagnosis for SMZL includes several other conditions that can mimic its clinical presentation.
Key Differential Diagnoses:
- Hairy Cell Leukaemia (HCL): HCL is a rare type of cancer that affects the blood and bone marrow, often presenting with splenomegaly and lymphocytosis. It is more common in people who have been infected with hepatitis C virus and some autoimmune conditions [5].
- Splenic Diffuse Red Pulp Lymphoma: This is another rare subtype of non-Hodgkin lymphoma that can involve the spleen, making it a differential diagnosis for SMZL.
- Follicular Lymphoma (grades 1 and 2): Follicular lymphoma is a type of non-Hodgkin lymphoma that can selectively involve the splenic red pulp, making it a differential diagnosis for SMZL [6].
- Mantle Cell Lymphoma: Mantle cell lymphoma is another rare subtype of non-Hodgkin lymphoma that can involve the spleen, making it a differential diagnosis for SMZL.
Diagnostic Considerations:
The diagnosis of SMZL requires either spleen histology or, alternatively, the documentation of a typical cell morphology and immunophenotype on blood cells [1]. The differential diagnoses considered in this case were SMZL, Hairy Cell Leukaemia (HCL), Hairy Cell Leukaemia – Variant (HCL-V), Splenic Diffuse Red Pulp Lymphoma, and others [4].
References:
[1] Apr 28, 2016 — Diagnosis requires either spleen histology or, alternatively, the documentation of a typical cell morphology and immunophenotype on blood cells ...
[2] Jan 12, 2024 — Splenic marginal zone lymphoma (SMZL) is an uncommon subtype of non-Hodgkin lymphoma that usually presents with splenomegaly and lymphocytosis ...
[3] by M Mollejo · 2020 · Cited by 2 — Marginal zone lymphoma differential diagnosis is based on an integration of clinical, morphological and molecular features.
[4] by A Mohanpuria · 2017 · Cited by 2 — The differential diagnoses considered in this case were SMZL, Hairy Cell Leukaemia (HCL), Hairy Cell Leukaemia – Variant (HCL-V), Splenic Diffuse Red Pulp Lymphoma, and others.
[5] More common in people who have been infected with hepatitis C virus and some autoimmune conditions.
[6] Follicular lymphoma is a type of non-Hodgkin lymphoma that can selectively involve the splenic red pulp.
Additional Differential Diagnoses
- obsolete lymphoid leukemia
- obsolete leukemic reticuloendotheliosis of intrathoracic lymph nodes
- Hairy Cell Leukaemia (HCL)
- Splenic Diffuse Red Pulp Lymphoma
- mantle cell lymphoma
- follicular lymphoma
Additional Information
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