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A 17-year-old girl presented to a hospital orthopedics department in Vietnam due to pain in her left hip joint, which she noted had been getting worse over the past month. Neither her personal nor her past medical history offered any diagnostic clues, and she did not have any relevant family history.

However, she explained that she had been hurt in a car accident the previous year, and although she received treatment at a local hospital at the time, her hip had been bothering her since then. Neither her diagnosis nor the treatment she received at that time had been documented, although she said that she had not received surgery for the injury.

Examination in the current hospital identified limited range of motion in the left hip, and the patient was admitted. Computed tomography (CT) scan revealed a lytic bone lesion in the wing (ala) of the left ilium, and the bone lesion was then biopsied.

Histopathologic examination suggested a diagnosis of plasmacytoma or multiple myeloma, and the patient was referred to a blood transfusion and hematology hospital. At that time, the patient required crutches for walking.

Examination showed no evidence of hemorrhage, infection, or enlargement of the spleen, liver, or lymph glands. Results of cardiovascular and pulmonary examinations were unremarkable, and her vital signs were normal.

Upon further assessment, the patient was diagnosed with immunoglobulin (Ig) G lambda multiple myeloma, stage IIIA, according to the , or stage I disease according to the for myeloma, and medium-risk disease.

X-ray and CT scan showed several lytic lesions of the left pubis and wing of ilium. Positron emission tomography scan identified several lytic bone lesions, hyperattenuation of the femoral bone, and increased metabolic activity.

The patient was started on induction chemotherapy with bortezomib (Velcade; 1.3 mg/m² on days 1, 4, 8, and 11) and dexamethasone (40 mg on days 1-4 and days 8-11) every 3 weeks for four cycles.

She then underwent autologous stem cell transplant (ASCT) with high-dose melphalan (200 mg/m²), followed by another two cycles of bortezomib and dexamethasone.

Laboratory findings at baseline and after ASCT were as follows:

• Hemoglobin: 10.9 g/dl (baseline), 12.3 g/dl (post-ASCT)

• Platelet count: 214 k/ul, 240 x 10³/μl

• White blood cell count: 5.54 k/ul, 7.5 x 10³/μl

• β2 microglobulin: 1.49 mg/L, 1.26 mg/L

• Serum quantitative IgG: 41.73 g/L (7-16), 8.42 g/L

• Serum protein electrophoresis, albumin/globulin: 0.66, 1.31

• Proteinuria of Bence Jones in urine: Negative at both time periods

• Bone marrow aspirate, plasma cells: 7%, 3%

• Bone marrow biopsy, plasma cell: <5%, <5%

• Fluorescence in situ hybridization: No abnormality detected at either time

The patient was also treated with bisphosphonates at the beginning of every cycle, and given pain medication as needed. She started physical therapy to help address the need for crutches, and preventive treatment with acyclovir and trimethoprim/sulfamethoxazole was initiated.

She reported one adverse event of constipation, which began and resolved without intervention during the first cycle of chemotherapy. After completing induction with four cycles of bortezomib and dexamethasone, she achieved a very good partial response (VGPR), her pain completely subsided, and she was able to start walking without crutches.

Clinicians performed x-rays after treatment, which showed that the bone mass was reduced in size, which was also confirmed in a follow-up CT scan.

Furthermore, the levels of gamma globulins had fallen to almost normal levels from the elevated levels noted prior to treatment, and immuno-electrophoresis showed that the IgG lambda component decreased after treatment.

After completing ASCT and undergoing consolidation therapy with two cycles of bortezomib and dexamethasone, the patient continued to respond well with a VGPR, and she remained mobile.

She and her family opted to receive thalidomide (100 mg/day) as maintenance therapy, and she remained clinically stable at the 14-month follow-up.

Discussion

The authors presenting this very rare of an atypical presentation of multiple myeloma in a 17-year-old girl noted that however rare the incidence, physicians should be aware of the possibility of multiple myeloma in this scenario.

And while myeloma overall represents about of all hematologic malignancies, multiple myeloma is comparatively rare in Vietnam, where this case occurred, with incidence of 530 new cases per year and a 5-year prevalence of 1,093 individuals, the authors noted.

They cited from the NCI Surveillance, Epidemiology, and End Results (SEER) program, which "recently reported an incidence between 20-34 years, 35-44 years, 45-54 years, and 55-64 years of 0.5%, 2.7%, 10.6%, and 23.2%, respectively, with no reported cases in patients under 20 years of age," the case authors wrote.

Other highlights from the SEER analysis:

• More men than women are affected

• Multiple myeloma develops more often in people ages 65 to 74

• Median age at diagnosis is 69

• As of 2012-2016, the rate of new cases per 100,000 was 8.7 for men and 5.6 for women

In addition, multiple myeloma is similar across groups in terms of the clinical characteristics and response to treatment. The case authors noted that there are occasional reports of multiple myeloma presenting similarly in other younger patients, with one involving the skull and ribs in a 23-year-old woman, with another presenting as solitary plasmacytoma of the tibia in a 21-year-old man.

A 2008 of 10,549 patients by the International Myeloma Working Group found that patients younger than 40 were more frequently male, with more favorable features such as low International Staging System and Durie-Salmon stage, as well as less often having adverse prognostic factors such as high C-reactive protein, low hemoglobin, increased serum creatinine, and poor performance status.

That analysis reported a median overall survival of about 14 years among 52 myeloma patients diagnosed up to age 30 (age range, 8-30), and prognosis in young patients appeared to be at least as good as if not better than that of myeloma patients overall, possibly because of the use of novel agents and hematopoietic stem cell transplantation in younger patients, the case authors suggested.

A 2016 multi-institutional of 173 patients ages 21-40 and 916 ages 41-60 between 2000 and 2015 reported that 5-year rates of overall survival in younger versus older patients were 83% versus 67%, while 10-year rates were 56% versus 39% (P<0.001), and the results among the subgroup of 780 patients who underwent autologous transplantation were similar.

For the 17-year-old patient in the current case report, treatment with ASCT was associated with a VGPR that was sustained for more than a year. At that time, antimyeloma agents available in Vietnam included bortezomib, dexamethasone, cyclophosphamide, and thalidomide, the authors noted.

The accessibility and affordability of treatment, along with patient adherence to therapeutic recommendations, are commonly encountered challenges when managing myeloma there, the team added, which is also why generic bortezomib was chosen in this patient's case due to cost considerations.

The case authors also noted that because induction with bortezomib and thalidomide can increase the risk of developing peripheral neuropathy, patients may often have to discontinue therapy, but because this patient was young and female, an effective and well-tolerated of bortezomib and dexamethasone was chosen.

The only adverse effect of treatment in this patient was constipation, which developed during the first cycle and resolved without treatment, the case authors noted. In addition, the patient had a VGPR after induction, including improvement of her hip pain and mobility. Subsequently, consolidation therapy with two cycles of bortezomib and dexamethasone was provided, and the response was maintained 14 months later.

Clinicians noted that they counseled the patient and her family about the importance of maintenance therapy. For financial and insurance reasons, and to reduce the frequent trips to the hospital that would be needed for bortezomib injections, the family chose thalidomide maintenance after a discussion of the risks of fetal teratogenicity associated with thalidomide.

Conclusion

The authors concluded that this case illustrates an atypical presentation of myeloma, but however rare, the possibility is something that physicians should be aware of, to increase the chances of early diagnosis and optimum treatment. The outcome in this case indicates that a generic bortezomib-based regimen coupled with stem cell transplantation is a good option in terms of both response and cost, the authors stated.

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