Β2 MICROGLOBULIN. ON THE CLUE OF A LYMPHOPROLIFERATIVE DISEASE.

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Transcripción de la presentación:

Β2 MICROGLOBULIN. ON THE CLUE OF A LYMPHOPROLIFERATIVE DISEASE. Hospital Universitario de Canarias. Tenerife. Β2 MICROGLOBULIN. ON THE CLUE OF A LYMPHOPROLIFERATIVE DISEASE. UVEITIS SECTION ROCHA CABRERA P, DE AGOSTINO RGUEZ J, LOSADA CASTILLO MJ, RODRIGUEZ LOZANO B, LOZANO LÓPEZ V, SERRANO GARCÍA MA 1

R.E CASE REPORT R.E Female 73 y.o. P.H: MD type 2. Hypertension. O.H: not previous AAU. No previous surgeries. Loss and progressive bilateral visual acuity. VA 0.2 LE 0.1 RE. PIO (G) 14/14 mmHg L.E L.E

R.E L.E NO VITRITIS DIFFICULT EVALUATION BECAUSE SYNECHIAE 429 micras OD, 440 OI (éste último es aproximado). La catarata es mayor en el OI por eso se ve más turbio con respecto que el contralateral. Bilateral Tenon triamcinolone acetonide Topically prednisone decreasing dose + cycloplegic. 3

SYSTEMIC STUDY ANALYSIS: Normal blood count, ESR 59 mm / h, CRP 13.7 mg / L, ACE 10, beta-2 microglobulin 3.94. VDRL negative.

1 month: V.A. 0.5 RE, 0.25 LE B.M.C: RE no activity. PK. LE no activity. PK persist IOP (G) 18/16 mmHg. No vitritis b.e. RE Refiere pérdida de 8 kg de peso en 6 meses LE OZURDEX® IV BECAUSE NO IMPROVEMENT AND WORSE VA 8 kg loss in 6 months 5

PCR / ESR / β 2 microglobulin is again requested. Abdominal and pelvic ECO were done, to assess spleen and retroperitoneal lymph nodes, "suggestive of mesenteric panniculitis". PCR / ESR / β 2 microglobulin is again requested. Results: VSG 44, α-globulin 14.4, 14.44 PCR, β 2 microglobulin 3.75 ABDOMINAL/PELVIC/CHEST TAC Globulinas alfa 1 Artículo principal: Alfa globulina La alfa 1 o antitripsina: Es la encargada de controlar la acción de las enzimas lisosomales. Nota: es reactante de fase aguda. La TBG: Se encarga de fijar la hormona tiroidea. Transporta T3 y T4. La alfa 1 glucoproteína ácida: También conocida como orosomucoide, es un reactante de fase aguda sintetizado en el hígado como respuesta a la inflamación y daño del tejido. La RBP: Es la hormona fijadora de retinol, la cual transporta vitamina "A". Normalmente se asocia a la prealbúmina. Globulinas alfa 2[editar] El grupo de las globulinas alfa 2 está compuesto por las siguientes: La macroglobulina (alfa 2): La función primordial de ésta es neutralizar las enzimas proteolíticas. La haptoglobina: Es la encargada de fijar la Hb plasmática de los eritrocitos, y la transporta al hígado para que no se excrete por la orina. La ceruloplasmina: Transporta y fija el 90 por ciento del cobre sérico. La eritropoyetina: Sintetizada en el riñón ante una hipoxemia, es la resposable de la formación de eritrocitos y plaquetas. Los valores normales de la beta dos son hasta 3 mg/dl. 2 MONTHS VA RE 0.4 LE 0.15 CATARACT LE VA RE 0.5 VA LE 0.3 FEC + IOL 6

ABDOMINAL TAC

TORAX TAC Multiple mediastinal lymphadenopathy and lower right paratracheal, subcarinal / paraesophageal. Bilateral hilar lymphadenopathy and lower interlobes. 8

LYMPHADENOPATHY + β 2 MICROGLOBULIN WE MUST RULE OUT LYMPHOPROLIFERATIVE SYNDROME LYMPH NODE BIOPSY EUS-FNA ENOUGH MATERIAL

GRANULOMAS SARCOID-LIKE Thoracic Surgery: Biopsy of nodes RESULTS OF PATHOLOGY GRANULOMAS SARCOID-LIKE SARCOIDOSIS

CONCLUSIONS Β2- lymphoproliferative disease was the first option, but there are other causes that we must consider. Our case oriented towards lymphoproliferative disease and ECA negative. ECA negative does not rule out sarcoidosis. The patology has been essential in the diagnosis after lymph node biopsy. The β2 in our case was crucial because this finding of elevation, abdominal ECO led to TAC and finally diagnosed after biopsy. First possibility for thinking as lymphoma.