We present the case of a 32-year-old male, with no known drug allergies, with the only personal history of polyarticular rheumatism in childhood. He was admitted from the emergency department for severe pain in the left hemithorax, which did not change with respiratory movements or postural changes, without productive cough or dyspnoea, and with no other accompanying symptoms.
Physical examination revealed a small non-specific right laterocervical lymphadenopathy, with a hepatomegaly of about 3 cm from the costal ridge, with no other findings of interest.
The patient presented the following complementary tests:
BASIC COMPLEMENTARY TESTS
Blood count and peripheral blood smear: haemoglobin 13.3 g/dl, platelets 281,000/ul, leukocytes 8,800/ul with 23% segmented, 1% segmented, 1% metamyelocytes, 2% monocytes, 33% lymphocytes (most of them of plasmacytoid appearance), and 40% plasma cells, some of which are large binucleated.
Biochemistry: Creatinine 1.2 mg/dl, urea 52 mg/dl, (creatinine clearance 59 ml/min), total protein 10.8 g/dl, total calcium 9.8 mg/dl, rest normal.
Proteinogram: monoclonal peak in gamma band.
Coagulation: APTT 33.6 seconds, INR 1.35.
Basic urine test: Protein positive, with presence of mucin in the sediment.
Chest X-ray: small calcified nodular image in left lower lobe, probable calcified granuloma.
SPECIFIC COMPLEMENTARY TESTS
Peripheral blood immunophenotype: 45% of the total leukocyte count shows an abnormal immunophenotype: CD45+, CD38+, CD138+, CD56+, CD19-, CD20-, CD5, CD10-, CD23-, Cd79b-, sIg-.
Bone marrow aspirate: Presence of plasma cells in a proportion of 50% of the total cellularity, of heterogeneous size with predominantly intermediate-sized elements. Binucleated and trinucleated cells are frequent.
Bone marrow biopsy: Multifocal interstitial infiltration of the bone marrow parenchyma by plasmablastic IG-Kappa plasma cells. Marrow plasmacytosis of 40-50%. Negative amyloid material.
Beta2-microglobulin: 7.3 mg/dl.
Bone series: No evidence of osteolytic lesions.
Serum immunoelectrophoresis: IgG 4.390 mg/dl, IgA 14.5mg/dl, IgM 4.17 mg/dl. Serum immunofixation: Paraprotein IgG-Kappa.
Urine immunoelectrophoresis: Light chain proteinuria (Bence-Jones) of 1,310 mg in 24 hours, for IgG-Kappa paraprotein and free Kappa light chains.
Serology HBV, HCV, HIV: negative.
Abdominal ultrasound: hepatomegaly, without focal hepatosplenic lesions. There was no evidence of intra-abdominal adenopathy or free intraperitoneal fluid, the rest was unaltered.
Diagnosis: The patient presented a picture compatible with multiple myeloma in its primary plasma cell leukaemia variant.
Treatment: Given the patient's age, an autologous peripheral blood progenitor cell transplant was decided. For which induction chemotherapy was started with the C-VAD scheme.
