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A patient with splenic marginal zone lymphoma presenting with spastic paraplegia as the initial symptom.

Wada Y, Nishimura Y, Hashimoto K - Case Rep Neurol (2011)

Bottom Line: Abdominal computed tomography images showed moderate splenomegaly.Abnormal lymphocytes of B-cell lineage markers (CD19+, CD20+, and CD25+; surface immunoglobulin κ expression; IgD+ and IgM+) were found in the peripheral blood, cerebrospinal fluid, bone marrow and spleen.After the completion of chemotherapy, the patient was in complete remission, and spinal MRI findings were normal.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, Nishi-Kobe Medical Center, Kobe, Japan.

ABSTRACT
In this report, we describe the case of a patient with splenic marginal zone lymphoma (SMZL) who presented with spastic paraplegia as the initial symptom. A 42-year-old male developed progressive spastic paraplegia over 4 months. His neurologic examination revealed paraplegia with pyramidal syndrome, hypoesthesia below the T1 level, and anal hypotonia. Magnetic resonance imaging (MRI) of the spinal cord revealed an extensive high-intensity signal in T2-weighted sequences and swelling involving the thoracic region and conus medullaris. A laboratory test revealed presence of the serum M component. Abdominal computed tomography images showed moderate splenomegaly. Abnormal lymphocytes of B-cell lineage markers (CD19+, CD20+, and CD25+; surface immunoglobulin κ expression; IgD+ and IgM+) were found in the peripheral blood, cerebrospinal fluid, bone marrow and spleen. Splenectomy confirmed the SMZL diagnosis. After the completion of chemotherapy, the patient was in complete remission, and spinal MRI findings were normal. Intramedullary spinal cord involvement in SMZL is extremely rare, and, to the best of our knowledge, this is the first case of SMZL with intramedullary spinal cord involvement associated with clinical and radiologic signs without the involvement of cerebral structures. Spastic paraplegia can be the initial presentation of SMZL.

No MeSH data available.


Related in: MedlinePlus

a Abdominal CT scan showing moderate splenomegaly. b Spleen section showing prominent white pulp infiltration by lymphocytes with marginal zone expansion (HE staining; original magnification ×40).
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Figure 2: a Abdominal CT scan showing moderate splenomegaly. b Spleen section showing prominent white pulp infiltration by lymphocytes with marginal zone expansion (HE staining; original magnification ×40).

Mentions: A 42-year-old male patient with no history of illness was admitted to our hospital because of progressive spastic paraplegia over 4 months. In November 2004, he noticed paresthesia in the soles of his feet. In February 2005, he developed paresthesia in both hands, spasticity of his legs, and paresthesia below the T10 level. Over the next few months, he noted a progressive worsening of spastic paraplegia and sensory disturbance at the T1 sensory level. On admission, neurologic examinations revealed paraplegia with pyramidal syndrome, hypoesthesia below the T1 level, and anal hypotonia. Other examinations showed normal results. WBC count was 5,700/dl, with 46% lymphocytes (19% of which were atypical). Viral and bacterial test results and the LDH level were normal. Serum protein electrophoresis revealed the presence of an IgM paraprotein. The level of a soluble anti-interleukin-2 receptor antibody was elevated (3,830 U/ml). The nerve conduction study yielded normal results. Spinal cord magnetic resonance imaging (MRI) revealed an extensive high-intensity signal in T2-weighted sequences and swelling involving the thoracic region (from T1 to T12) and conus medullaris (fig. 1). There was no gadolinium enhancement in T1-weighted sequences. Brain MRI findings were normal. Cerebrospinal fluid (CSF) examination showed a high protein level with atypical lymphocytes. Flow cytometry of peripheral blood and CSF showed B-lineage markers (CD19+, CD20+, and CD25+; surface immunoglobulin κ expression; IgD+ and IgM+, CD5- and CD103-). Abdominal computed tomography (CT) images showed moderate splenomegaly (fig. 2). No peripheral lymphadenopathy or hepatomegaly was detected. Splenectomy was performed. A histopathological analysis of the spleen showed typical white pulp lymphocyte infiltration (fig. 2). The lymphoid cells exhibited the same phenotypic B-lymphocyte infiltration as in the peripheral blood, CSF, and bone marrow. The diagnosis of SMZL was established. Intrathecal cytarabine, methotrexate, hydrocortisone sodium phosphate, systemic fludarabine phosphate, and rituximab were administered.


A patient with splenic marginal zone lymphoma presenting with spastic paraplegia as the initial symptom.

Wada Y, Nishimura Y, Hashimoto K - Case Rep Neurol (2011)

a Abdominal CT scan showing moderate splenomegaly. b Spleen section showing prominent white pulp infiltration by lymphocytes with marginal zone expansion (HE staining; original magnification ×40).
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC3064864&req=5

Figure 2: a Abdominal CT scan showing moderate splenomegaly. b Spleen section showing prominent white pulp infiltration by lymphocytes with marginal zone expansion (HE staining; original magnification ×40).
Mentions: A 42-year-old male patient with no history of illness was admitted to our hospital because of progressive spastic paraplegia over 4 months. In November 2004, he noticed paresthesia in the soles of his feet. In February 2005, he developed paresthesia in both hands, spasticity of his legs, and paresthesia below the T10 level. Over the next few months, he noted a progressive worsening of spastic paraplegia and sensory disturbance at the T1 sensory level. On admission, neurologic examinations revealed paraplegia with pyramidal syndrome, hypoesthesia below the T1 level, and anal hypotonia. Other examinations showed normal results. WBC count was 5,700/dl, with 46% lymphocytes (19% of which were atypical). Viral and bacterial test results and the LDH level were normal. Serum protein electrophoresis revealed the presence of an IgM paraprotein. The level of a soluble anti-interleukin-2 receptor antibody was elevated (3,830 U/ml). The nerve conduction study yielded normal results. Spinal cord magnetic resonance imaging (MRI) revealed an extensive high-intensity signal in T2-weighted sequences and swelling involving the thoracic region (from T1 to T12) and conus medullaris (fig. 1). There was no gadolinium enhancement in T1-weighted sequences. Brain MRI findings were normal. Cerebrospinal fluid (CSF) examination showed a high protein level with atypical lymphocytes. Flow cytometry of peripheral blood and CSF showed B-lineage markers (CD19+, CD20+, and CD25+; surface immunoglobulin κ expression; IgD+ and IgM+, CD5- and CD103-). Abdominal computed tomography (CT) images showed moderate splenomegaly (fig. 2). No peripheral lymphadenopathy or hepatomegaly was detected. Splenectomy was performed. A histopathological analysis of the spleen showed typical white pulp lymphocyte infiltration (fig. 2). The lymphoid cells exhibited the same phenotypic B-lymphocyte infiltration as in the peripheral blood, CSF, and bone marrow. The diagnosis of SMZL was established. Intrathecal cytarabine, methotrexate, hydrocortisone sodium phosphate, systemic fludarabine phosphate, and rituximab were administered.

Bottom Line: Abdominal computed tomography images showed moderate splenomegaly.Abnormal lymphocytes of B-cell lineage markers (CD19+, CD20+, and CD25+; surface immunoglobulin κ expression; IgD+ and IgM+) were found in the peripheral blood, cerebrospinal fluid, bone marrow and spleen.After the completion of chemotherapy, the patient was in complete remission, and spinal MRI findings were normal.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, Nishi-Kobe Medical Center, Kobe, Japan.

ABSTRACT
In this report, we describe the case of a patient with splenic marginal zone lymphoma (SMZL) who presented with spastic paraplegia as the initial symptom. A 42-year-old male developed progressive spastic paraplegia over 4 months. His neurologic examination revealed paraplegia with pyramidal syndrome, hypoesthesia below the T1 level, and anal hypotonia. Magnetic resonance imaging (MRI) of the spinal cord revealed an extensive high-intensity signal in T2-weighted sequences and swelling involving the thoracic region and conus medullaris. A laboratory test revealed presence of the serum M component. Abdominal computed tomography images showed moderate splenomegaly. Abnormal lymphocytes of B-cell lineage markers (CD19+, CD20+, and CD25+; surface immunoglobulin κ expression; IgD+ and IgM+) were found in the peripheral blood, cerebrospinal fluid, bone marrow and spleen. Splenectomy confirmed the SMZL diagnosis. After the completion of chemotherapy, the patient was in complete remission, and spinal MRI findings were normal. Intramedullary spinal cord involvement in SMZL is extremely rare, and, to the best of our knowledge, this is the first case of SMZL with intramedullary spinal cord involvement associated with clinical and radiologic signs without the involvement of cerebral structures. Spastic paraplegia can be the initial presentation of SMZL.

No MeSH data available.


Related in: MedlinePlus