HOSPITAL VIRGEN ARRIXACA
Contributions
Type: Publication Only
Background
Light chain deposition disease (LCDD) is a rare monoclonal gammopathy characterized by the systemic deposition of non-amyloid immunoglobulin light chains causing a progressive accumulation of extracellular material with varying degrees of organ impairment. Although LCDD mainly affects the kidneys (it manifests as proteinuria and/or renal insufficiency) the involvement of other organs (e.g., heart, central nervous system and peripheral nerves) is very common. We present the case of a patient with a newly diagnosed cerebral restricted λ LCDD.
Aims
We present the case of a patient with a newly diagnosed cerebral restricted λ LCDD.
Methods
Case report
A 49 -year-old female was admitted to our hospital with progressive loss of coordination in her right hand and some in her left leg for 3 months, associated with mayor depression, headaches and constitutional symptoms (asthenia, weight loss and anorexia). Clinical examination revealed a decrease in the strength and the sensitivity in the right hemisphere 4/5. Her complete blood count showed a mild anaemia (Hb 114 g/L) and trombocytopenia (122.000/uL) with total leukocyte in the normal range. Calcium was normal. Liver and renal function was also normal. Serum and urine protein electrophoresis did not reveal any monoclonal protein spike. Immunofixations were negative. Nephelometry was performed showing a decreased of IgA and IgM levels with a normal IgG. Serum λ free light chains were 6.98 mg/L with a normal or K/λ ratio (K/λ:). The MRI study was made to the patient in order to discard a central nervous system (CNS) neoplasm. The MRI revealed a space occupying diffuse lesion in left front parietal. Histological examination with hematoxylin and eosin of the resected tumor demostrated an amorphous eosinophilic deposits in white and grey matter of the brain without plasmacytic cells. These deposits were positive for λ-light chain antibody by immunohistochemistry. To rule out other plasma cell dyscrasias, bone marrow aspiration (BMA) and fine-needle aspiration of abdominal fat pad (FPFNA) were assessed. BMA showed a 3% of plasma cells (PC). Flow cytometry did not evidence monoclonal PC. FPFNA was negative to amyloid. With these data, Multiple Myeloma (MM) and Primary Systemic Amyloidosis (PSA) were discarded and the diagnosis of λ cerebral LCDD was made. The patient received conditioning regimen consisted of BEAM: BCNU 300 mg/m2 (d-6) cytosine arabinoside 200 mg/m2/d and VP-16 200 mg/m2/d (d-5 to d-2) and melphalan 200 mg/m2 (d-1) followed by autologous stem cell transplantation (ASCT). Currently (After 3 months of ASCT) the patient has been improved her strength and sensitivity.
Results
Discussion
LCDD is a rare monoclonal gammopathy and it is less common involving CNS. As in other neurological disorders, the clinical presentation (epilepsy, cognitive impairments, hemiparesis,headaches.) depends mainly on the location of deposition and not on the histological finding. Although chemotherapy including an alkylating agent, radiotherapy or steroids have been reported in a few cases, the standard treatment has not yet been clearly established. ASCT has been reported as a feasible strategy in LCDD. We choised BEAM conditioning regimen in order to cross the blood-brain barrier. The median overall survival is approximately of 4 years. Prognostic factors for LCDD include age, presence of concomitant MM or PSA. Nowadays, there is not enough data about the management of brain LCDD and more information is needed describing the efficacy of the different treatments.
Summary
We choised BEAM conditioning regimen in order to cross the blood-brain barrier. The median overall survival is approximately of 4 years. Prognostic factors for LCDD include age, presence of concomitant MM or PSA. Nowadays, there is not enough data about the management of brain LCDD and more information is needed describing the efficacy of the different treatments.
Keyword(s): Myeloma
Session topic: Publication Only
Type: Publication Only
Background
Light chain deposition disease (LCDD) is a rare monoclonal gammopathy characterized by the systemic deposition of non-amyloid immunoglobulin light chains causing a progressive accumulation of extracellular material with varying degrees of organ impairment. Although LCDD mainly affects the kidneys (it manifests as proteinuria and/or renal insufficiency) the involvement of other organs (e.g., heart, central nervous system and peripheral nerves) is very common. We present the case of a patient with a newly diagnosed cerebral restricted λ LCDD.
Aims
We present the case of a patient with a newly diagnosed cerebral restricted λ LCDD.
Methods
Case report
A 49 -year-old female was admitted to our hospital with progressive loss of coordination in her right hand and some in her left leg for 3 months, associated with mayor depression, headaches and constitutional symptoms (asthenia, weight loss and anorexia). Clinical examination revealed a decrease in the strength and the sensitivity in the right hemisphere 4/5. Her complete blood count showed a mild anaemia (Hb 114 g/L) and trombocytopenia (122.000/uL) with total leukocyte in the normal range. Calcium was normal. Liver and renal function was also normal. Serum and urine protein electrophoresis did not reveal any monoclonal protein spike. Immunofixations were negative. Nephelometry was performed showing a decreased of IgA and IgM levels with a normal IgG. Serum λ free light chains were 6.98 mg/L with a normal or K/λ ratio (K/λ:). The MRI study was made to the patient in order to discard a central nervous system (CNS) neoplasm. The MRI revealed a space occupying diffuse lesion in left front parietal. Histological examination with hematoxylin and eosin of the resected tumor demostrated an amorphous eosinophilic deposits in white and grey matter of the brain without plasmacytic cells. These deposits were positive for λ-light chain antibody by immunohistochemistry. To rule out other plasma cell dyscrasias, bone marrow aspiration (BMA) and fine-needle aspiration of abdominal fat pad (FPFNA) were assessed. BMA showed a 3% of plasma cells (PC). Flow cytometry did not evidence monoclonal PC. FPFNA was negative to amyloid. With these data, Multiple Myeloma (MM) and Primary Systemic Amyloidosis (PSA) were discarded and the diagnosis of λ cerebral LCDD was made. The patient received conditioning regimen consisted of BEAM: BCNU 300 mg/m2 (d-6) cytosine arabinoside 200 mg/m2/d and VP-16 200 mg/m2/d (d-5 to d-2) and melphalan 200 mg/m2 (d-1) followed by autologous stem cell transplantation (ASCT). Currently (After 3 months of ASCT) the patient has been improved her strength and sensitivity.
Results
Discussion
LCDD is a rare monoclonal gammopathy and it is less common involving CNS. As in other neurological disorders, the clinical presentation (epilepsy, cognitive impairments, hemiparesis,headaches.) depends mainly on the location of deposition and not on the histological finding. Although chemotherapy including an alkylating agent, radiotherapy or steroids have been reported in a few cases, the standard treatment has not yet been clearly established. ASCT has been reported as a feasible strategy in LCDD. We choised BEAM conditioning regimen in order to cross the blood-brain barrier. The median overall survival is approximately of 4 years. Prognostic factors for LCDD include age, presence of concomitant MM or PSA. Nowadays, there is not enough data about the management of brain LCDD and more information is needed describing the efficacy of the different treatments.
Summary
We choised BEAM conditioning regimen in order to cross the blood-brain barrier. The median overall survival is approximately of 4 years. Prognostic factors for LCDD include age, presence of concomitant MM or PSA. Nowadays, there is not enough data about the management of brain LCDD and more information is needed describing the efficacy of the different treatments.
Keyword(s): Myeloma
Session topic: Publication Only