One Case of POMES Syndrome with Limb Pain As the Main Presentation

Case Report

Austin Med Sci. 2022; 7(3): 1070.

One Case of POMES Syndrome with “Limb Pain” As the Main Presentation

Wang L¹, Fan Y¹, Zhang H¹* and Dong X²

¹Department of Neurology, Liaocheng People’s Hospital, Shandong Province, China

²Department of Clinical Medicine, Qingdao University, Qingdao City, Shandong Province, 266000, Qingdao City, Shandong Province

*Corresponding author: Huiyuan Zhang, Department of Neurology, Liaocheng People’s Hospital, Shandong Province, Liaocheng City, Shandong Province, 252000, China

Received: September 26, 2022; Accepted: October 28, 2022; Published: November 04, 2022


Objective: To summarize the clinical manifestations, imaging features and gene mutation sites of a patient with pomes syndrome with “limb pain” as the main manifestation, so as to improve the understanding of this rare disease.

Medical History: The patient was a 34 year old male with limb pain and fatigue for more than 20 days.

Symptoms and Signs: The tongue is in the middle, the uvula is in the middle, the bilateral pharyngeal reflex disappears, the neck is soft, the muscle strength of both upper limbs is grade 5, the proximal muscle strength of both lower limbs is grade 4, and the distal muscle strength is grade 5. The muscle tension of the extremities is normal, the tendon reflex of the extremities disappears, and no obvious abnormalities are found in the examination of deep and shallow sensation and ataxia.

The diagnostic method bone marrow puncture showed thrombocytosis. The pathological results showed that the hematopoietic cells of the three lines were proliferative, the oligoclonal bands in CSF were positive, and the oligoclonal bands in serum were positive. Serum immunofixation electrophoresis (March 30, 2018): IgG λ Abnormal monoclonal bands were found in the lane, and the type of monoclonal immunoglobulin was IgG- λ Type. Blood analysis suggested thrombocytosis (738 × 10°several L).

There is no specific therapeutic drug, but hormone and hydroxyurea are effective.

The clinical outcome was improved and the disease was gradually alleviated.

The clinical conclusion is that poems syndrome is a rare clinical syndrome with various forms. Early intervention is effective for the prognosis of patients.

Keywords: POMES syndrome; thrombocytosis; monoclonal plasma cells


Also known as Crow-Fukase syndrome, is a systemic lesion related to plasmaccytosis, mainly manifested as: polyneuropathy (polynel Jropath, P), organomegaly (Organmegaly, 0), endocrinopathy (Erldocfinopathy, E), M protein (M-protein, P) and skin lesions (Skin-change, S), etc. In 1980, Bardwic abbreviated the English prefix for the clinical features of the syndrome. The cause and principle of the disease are not very clear, and may be related to tumors, metabolic disorders, or abnormal immune response. One case of POMES syndrome with “limb pain” is mainly reported, aiming to improve the awareness of the disease, and reduce misdiagnosis and missed diagnosis.

Clinical Data

General Information

The 34-year-old male was admitted to hospital with “limb pain and weakness for over 20 days”. Patients more than 20 days ago no obvious cause of lower limb pain, knee upward, upper limbs pain is not obvious, gradually appear lower limbs fatigue, leg difficulties, mainly proximal, affect work, and facial, lower limbs swelling, then to our hospital, check blood analysis suggests thrombocytosis (73810 L), nuclear antibodies, immunoglobulin, complement tests, 14 days ago the patient to the provincial hospital, review platelets still elevated (81310 L), bone marrow puncture shows thrombocytosis, suggest to exclude reactive thrombocytosis considering myelocymoplastic tumor. Electromyography suggested multiple symmetric peripheral nerve damage, the patient was recommended to take oral “hydroxyurea and aspirin” treatment, the patient had oral traditional Chinese medicine treatment, the swelling symptoms were reduced, the pain was relieved, still felt limb fatigue, walking was “duck step”, for further clear diagnosis, we came to our hospital. Since the onset of the disease, the body temperature was high, obvious in the afternoon and evening, with the highest 38°C, and a recent weight loss of about 5kg. Night sweats” for 3 months, upper respiratory tract infection history of 2 months, has been cured. Denial history of hypertension, denied blood history, denied history of hepatitis tuberculosis. A history of trauma and allergy was denied. Denied cigarette smoking and alcohol consumption history.

Check Up; Inspect

Physical examination: Heart, lung and abdominal physical examination (-), superficial lymph nodes did not touch the enlargement. Ditting oedema in both ankles. Nervous system physical examination: mind, spirit, fluent speech, binocular ball movement flexible, no obvious nystagmus, bilateral nasolabial groove slightly symmetrical, extended tongue centered, udra, bilateral pharyngeal reflex disappear, the neck is soft, double upper limb muscle level 5, lower limb proximal muscle level 4, distal muscle level 5, four limb muscle tension is normal, limb tendon reflex disappear, deep feeling and mutual aid exercise examination no obvious abnormalities. Bilateral pathological signs (-), meningeal stimulation signs (-).

Auxiliary examination: prehospital immunoglobulin + complement, C opritin, anti-O, rheumatoid factor, antonuclear antibody, and anti-cyclic citrulline polypeptide antibody showed no abnormalities. Bone marrow puncture indicated thrombocytosis, and the pathological results (Figure 1) showed the hyperplasia of tertiary hematopoietic cells, with no significant increase in primitive cells and abnormal lymphocytes. The EMG suggests multiple symmetric peripheral nerve (root) damage. Cervical disc MR: C4 / 5 disc herniation with disc degeneration, cervical osteosis. Abdominal color ultrasound: indicating cholecystitis, no liver and spleen and other organomegaly. arteriovenous color ultrasound of both lower limbs: no abnormality.