Shi XX Male 36 years old Date of admission: 2019-02-19
Main complaint: pain and weakness of lower limbs with numbness of extremities for 2 months
History of present illness: Two months ago, the patient had pain and weakness in both lower limbs without obvious cause. It was aching, located below the knee joint, and appeared when walking and squatting. He went to the local county hospital for diagnosis of muscle and soft tissue injury and was treated with acupuncture, but no significant improvement. Later, he went to the Department of Neurology of the First Affiliated Hospital of Zhengzhou City. The electromyogram showed abnormal peripheral motor and peripheral sensory conduction functions of the limbs. The N20 conduction latency of the deep sensory conduction pathway of the upper limbs was delayed. The P40 conduction of the deep sensory conduction pathway of the two lower limbs was not induced. Fixed electrophoresis IgA-λ was positive, blood routine showed white blood cells 8.3*10^9/L, hemoglobin 160g/L, platelets 514*10^9/L, CT showed that T7 vertebral body bone destruction and high density shadow, splenomegaly , Vascular endothelial growth factor 689.8pg/ml, LH, PRL increased, TESTO decreased, considering POEMS syndrome, receiving MD program (melphalan 6mg / day * 15 days, dexamethasone 40mg once a week). For further treatment, he was admitted to the hospital with "POEMS syndrome". Since the onset of the onset, the patient has been in good spirits, poor sleep, good appetite, normal urination, normal stool, and no significant changes in weight.
Anamnesis: Hemorrhoid surgery was performed 4 years ago. Denies the history of other chronic diseases. Denies the history of smoking and drinking.
T 36.5℃ P 90 times/min R 15 times/min BP 130/80mmHg
Skin: The skin of the whole body becomes dark. The skin and mucous membranes had no rash, yellow staining, or bleeding spots. No liver palms, no spider moles, no edema. Lymph nodes: superficial lymph nodes are not palpable and enlarged.
Chest: Bilateral symmetrical, bilateral tactile tremor conduction is normal. No feeling of pleural friction, unvoiced sound in both lungs, clear breath sounds on both sides, no dry and wet rales and pleural friction sounds on both sides, breast development.
Heart: No bulge in the precordial area, normal apical pulsation, normal heart dullness, regular rhythm, no murmur in the auscultation area of each valve, no pericardial friction.
Abdomen: The abdomen is soft and flat. There is no gastrointestinal type and varicose veins in the abdominal wall, no tenderness and rebound pain. The liver and spleen are not under the ribs, Murphy's sign (-), drum sounds on percussion. There was no mobile dullness and vibrating sound, and bowel sounds 4 times per minute.
Limbs: No deformities of the limbs, no obvious abnormalities in joint movement and arterial pulsation of both upper and right lower limbs. No clubbing.
Nervous system: right lower limb muscle strength level Ⅱ, left lower limb muscle strength level Ⅲ, upper limb muscle strength level Ⅴ, physiological reflex exists, pathological reflex is not elicited.
Auxiliary examination (The First Affiliated Hospital of Zhengzhou University):
Electromyography: The peripheral motor and peripheral sensory conduction functions of the limbs are abnormal, the N20 conduction latency of the deep sensory conduction path of the upper limbs is delayed, and the P40 conduction of the deep sensory conduction path of the lower limbs is not led.
Abdominal ultrasound: splenomegaly,
Breast ultrasound: male breast development.
Reproductive hormones (2018-12-26): LH 9.34 mIU/mL↑ PRL 23.71 ng/mL↑ TESTO 1.56 ng/mL↓
Reproductive hormones (2019-01-24): PRL 24.33 ng/mL↑ E2 67.88 pg/mL ↑ TESTO 0.72 ng/mL ↓
Immunofixation electrophoresis IgA-λ positive
Vascular endothelial growth factor 689.8pg/ml
BRT: WBC 8.3 *10^9/L, Hb 160g/L, PLT 514*10^9/L
CSF (2018-12-25): IgG CSF 274 mg/L ↑, Alb CSF 706.5 mg/L ↑
Check after admission:
BRT: WBC 5.27 *10^9/L, Hb159g/L, PLT 201*10^9/L
White blood cell score: nS 81% ↑, L 13% ↓
Biochemical total items: TP 55.3 g/L ↓ ALB 37.2 g/L ↓ TG 3.2mmol/L ↑ AST 13U/L ↓, ALT 18U/L,
CK 14U/L ↓ LDH 114U/L ↓ BUN 4.92 mmol/L Cr 49.4umol/L ↓
Coagulation, CRP, ESR, 24h urine protein, β2-microglobulin (blood/urine): no obvious abnormalities
Serum protein electrophoresis: IgG 536 mg/dl ↓, κ light chain 461 mg/dl ↓, λ light chain 305 mg/dl ↓
Reproductive hormone: PRL 21.90 ng/mL ↑ E2 98.20 pg/mL ↑
A work: sTSH 8.51 uIU/ml ↑
2 times of bone penetration: 0.5% young pulp, 1.0% young pulp.
Flow cytometry: clonal plasma cells are visible.
Free light chain ratio 0.24:
Karyotype analysis: 46, XY
Immunofixation electrophoresis: no M component
EMG evoked potentials (2019-2-20): Abnormal somatosensory evoked potentials in limbs (peripheral).
Color Doppler ultrasound: left ventricular hypertrophy
Tricuspid regurgitation (mild)
Slightly increased pulmonary artery pressure (40mmHg)
Whole body low-dose CT bone scan:
Scapula, sternum, bilateral ribs, vertebral body, and pelvic bones in the scan area can be seen with multiple spot-like and nodular high-density shadows, some of which are puncture-like bone destruction foci, and some of the vertebral body and its cone facet joints are affected, with T7 vertebrae The bones are destroyed. Physiological curvature of the spine exists, and bone hyperplasia can be seen at the edges of some vertebral bodies.
There were no clear lesions in both lungs during the scan, and a small arc-shaped liquid density shadow was seen in the dorsal chest cavity on both sides, and a little more on the left side. The liver is enlarged.
Diagnosis: POEMS syndrome
1. The MP program is given to the outside hospital, but the effect is not good.
2. Current treatment:
PCD program *4 treatment, physical exercises, and planned peripheral blood autologous stem cell transplantation. At present, the skin color is obviously lighter, the breast glands have shrunk by more than half, the muscle strength of the lower limbs has returned to grade IV, the reproductive hormones have returned to normal, and VEGF has dropped by more than half.