Tariq Parvez, Abu Basher, Saleem Al Ahmadi, Matouga Baamer.
Chemotherapeutic response of patient with Plasmacytosis and Grave`s disease in Mixed Variant Multicentric Castleman`s Disease associated with POEMS Syndrome.
J Coll Physicians Surg Pak Jan ;12(11):711-4.

MHR, 32 years old male, black Mortician, heavy smoker of 11 years duration, with no diabetic or hypertensive history, was admitted on 30.3.2002. He complained of painless right inguinal swelling with progressively increasing weakness and heaviness associated with paraesthesia of both lower limbs of 3 years duration, and also similar problem in both upper limbs of 5 months duration. There was H/O of anorexia, weight loss of more than 15 kg, fever off and on and excessive sweating during the last 5 months. Prior to admission his weakness was so severe that he could not walk more than 50 meters and unable to get up from sitting position, also unable to carry out fine work by hands. Inguinal lymph node biopsy done on initial presentation showed features consistent with `Multicentric Castleman`s disease of Plasma cell type`. About 2 months prior to the above problems, he was detected to have Grave`s disease and was managed initially by B-blocker and Neomercazole and afterwards by Radioiodine 131. One month after the treatment he developed hypothyroidism and he was placed on thyroid replacement therapy. For seven years he has been having renal and ureteric stones bilaterally for which he was treated many times. He also complained of impotency for the last 3 years. One year back he was treated for polyneuropathy with steroids and got improved but his condition again deteriorated for the last 5 months. On examination there was bilateral moderate exophthalmos with moderate size diffuse goiter. There was bilateral lymphadenopathy of axilla and cervical region. He was having mild degree of bilateral gynaecomastia. There were excessive hair on both of his lower limbs. Cardiovascular and respiratory system were unremarkable. Abdominal examination showed mild hepatosplenomegaly. On CNS examination there was generalized muscle wasting which was more severe in all limbs including small muscles of the hands with decreased muscle tone. Left claw toes with wasted interosseous muscle were also noted. Deep tendon reflexes were lost in all limbs with equivocal planter response bilaterally. Bilaterally, there was marked weakness in knee extension, dorsiflexion of the ankles, but mild weakness in knee flexion, planterflexion of the ankles, flexion and extension of the hips. Severe weakness in extension of both elbows and small muscles of both hands was found. Mild weakness in elbow flexion and shoulder movement was found bilaterally. All modalities of sensations were diminished equally upto the mid thigh in lower limbs and upto mid arms in upper limbs except joint position sense in upper and lower limbs upto the level of mid arms and mid thighs. There was a mild wide based gait. On ophthalmoscopy both eyes showed papilledema with engorged veins. Superficial hemorrhages in left optic disc were found. On investigations blood CBC was within normal limits, except thrombocytosis. Blood biochemistry showed low plasma osmolality and serum sodium whereas blood sugar and LDH were high. Albumin and cholesterol were low but calcium was high in the blood. Immunoglobulin levels of IgA and IgM were normal but IgG level was three times higher in the blood. Serology for HIV, hepatitis and HHV-8 screen was negative. CSF examination was normal except low LDH and high glucose and protein. Blood level of prolactin and lutininzing hormone was raised, and that of testosterone was reduced. X-rays chest, skeletal survey, CT scan, ultrasound abdomen and pelvis, and IVP confirmed clinical findings and showed lymphadenopathy in axillary, mediastinal and pelvic regions along with hepatosplenomegaly and bilateral hydronephrosis with multiple stones. There were multiple rounded sclerotic bony metastatic lesions in spine and pelvic bones. A large expansile lytic lesion in the right iliac bone, with an adjacent large soft tissue mass and multiple pelvic nodes were seen. Bone marrow aspiration and Trephine biopsy showed moderate increase in plasma cells without lymphoma transformation. Excisional biopsy of the axillary lymph node done on this admission revealed `Castleman`s disease of hyaline vascular type`. Patient was placed on injection vinblastin weekly, tablet melphalan 5 mg twice daily for three days and prednisolone 20 mg three times daily for three days, to be repeated every 6 weeks.

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