POEMS

  • HBC vax

  • Period: to

    POEMS

  • first symptom: fever

  • arthralgias, asthenia, weight loss, fever

  • first ER: HCT 34, Hb 98, PLT 748

  • second ER: arthralgias, asthenia reduced Hb, increased PLT, normal CPK started steroid: methilprednisolone 80 mg, then tapering prednisone 25 mg

  • blood exams: Hb 117, PLT 942

  • third ER: fever, reduced mobility bed-seat Hb12, PLT 760

  • still complaining fever, acroparesthesias

  • Internal Medicine Admission; chest XR: accentuated the pulmonary pattern in the lower right perihilar area, which is associated with some tenuous radiopaque patches

  • neurological examination: reduced OTR, pain, non strenght or sensory defects

  • sloping edema of lower limbs; NF exam: prolonged F waves, absent H reflexes, reduced MCV; global spirometry: reduced alveolar-capillary CO diffusion

  • neurological examination: absent OTR, acroparestesias MGUS: 0.6 g/dL; increased beta2microglobulin; increased proBNP cardiac US: pericardial effusion

  • eye examination: no papilledema, posterior vitreous detachment

  • bone biopsy: neoplasm Lambda monoclonal plasma cell CD138+, Lambda+

    Megakaryocytes located singly or loosely aggregated, show variable dimensions and mild dysplasia notes. CD34+ blasts present in less than 5% of the bone marrow population. A portion of atypical plasmacytoid elements is found, 15-20% of the entire bone marrow population, CD138+, Lambda+ (CD56-, CD20-, Kappa -). amyloid (Thioflavin -) negative, compatible with neoplasm Lambda monoclonal plasma cell, with pleomorphic features.
  • total body PET: increased metabolic activity at the left deep gluteous area

  • tension headache

  • CT: right sigmoideal sinus thrombosis. Medial-basal pleural reaction, distelectic striae of bilateral parenchymal thickening. Pericardial effusion. Areas of bone sclerosis in the posterior arch of the left VIII rib and in the sternal body

    brain CT: right sigmoideal sinus thrombosis.
    whole body CT: Bilaterally posterior medial-basal pleural reaction with maximum thickness on the right around 6 mm. Concurrent striae and bands of parenchymal thickening in the bilateral mid-basal area of probable distelectic nature. Thin pericardial effusion. Area of bone sclerosis is recognizable in correspondence with the posterior arch of the VIII rib of the left and in the sternal body where it has a maximum diameter of 10 mm
  • Neurological ward admission

  • PLT 718

  • brain MRI: Thrombosis in the right sigmoid sinus. Not meningeal impregnation.PLT 541, beta2 micro globulin 4.23, 24h proteinuria 0.15, TSH 7.51, FT3 1.5, FT4 0.69, ATP 0, ATG 4.73

    blood exams: PLT 541, beta2 micro globulin 4.23, 24h proteinuria 0.15, TSH 7.51, FT3 1.5, FT4 0.69, ATP 0, ATG 4.73
    starting prednisone 25 mg, pregabalin, venlafaxine
    brain MRI: Thrombosis in the right sigmoid sinus that extends to the intracranial tract of the internal jugular, no other parenchymal changes in the supra- and sub-tentorial areas are evident. Not meningeal impregnation
  • CT: bortezomid, daracizumab

    CT: bortezomid, daracizumab