A case is reported of multiple myeloma in a 23-year-old man, including extensive clinical studies and postmortem examination. Multiple myeloma for this age group is considered to be extremely rare

Ma, C. K.

Maeda, K.

Rebuck, J. W.

Waldmann, R.

Henry Ford Health System Scholarly Commons

Henry Ford Hospital Medical JournalVolume 24 | Number 1 Article 43-1976Multiple myeloma in a 23-year-old man: A casereportC. K. MaK. MaedaR. WaldmannJ. W. RebuckFollow this and additional works at: https://scholarlycommons.henryford.com/hfhmedjournalPart of the Life Sciences Commons, Medical Specialties Commons, and the Public HealthCommonsThis Article is brought to you for free and open access by Henry Ford Health System Scholarly Commons. It has been accepted for inclusion in HenryFord Hospital Medical Journal by an authorized editor of Henry Ford Health System Scholarly Commons.Recommended CitationMa, C. K.; Maeda, K.; Waldmann, R.; and Rebuck, J. W. (1976) "Multiple myeloma in a 23-year-old man: A case report," Henry FordHospital Medical Journal : Vol. 24 : No. 1 , 19-26.Available at: https://scholarlycommons.henryford.com/hfhmedjournal/vol24/iss1/4H e n r y Fo rd H o s p . M e d . J o u r n a l V o l . 2 4 , N o . 1 , 1 9 7 6 Multiple myeloma in a 23-year-old man A case report C. K. M a , M D , * K. M a e d a , M D , * R. W a l d m a n n , D O * * and J. W. Rebuck, M D , PhD* M t A case is reported of multiple myeloma in a 23-year-old man, including extensive clinical studies and postmortem examina-tion. Multiple myeloma for this age group is considered to be extremely rare. * Department of Pathology ** Department of Medicine, Division of Hematology Address reprint requests to Dr. Koichi Maeda at Henry Ford Hospital, 2799 West Grand Boule-vard, Detroit, Ml 48202 u l t ip le m y e l o m a has been descr ibed in f requent ly in younger age groups. Its oc -cur rence in patients y o u n g e r t h a n 35 years of age is ex t remely rare. The present case is be ing reported because o f t h e y o u n g age of the pat ient w h o showed classical c l in ica l mani festat ions of mu l t i p le m y e l o m a . F ind-ings of the pos tmor tem examina t ion are con f i rmato ry . C a s e r e p o r t This 23-year-old single black male, a college student, was admitted to Henry Ford Hospital on March 20, 1973, after six weeks of heartburn, bloated feeling in the epigastrium after meals, and shortness of breath from the exertion of walking. Past history revealed that birth was normal and there was no significant illness in childhood. In 1960, at age 9, he had been admitted to Henry Ford Hospital because of ruptured appendix with generalized peritonitis. Physical examination for the current admission revealed a blood pressure of 160/80 mm Hg, pulse rate 130 per minute, regular, thyroid mildly enlarged, and no lymphadenopathy. The breasts were normal. The lungs were clear to percussion and auscultation. A grade II to grade VI systolic ejection murmur was heard over the left sternal border and there was a loud S-3 gallop at the left sternal border and at the base of the heart. The liver was palpable 15 cm below the right costal margin and was tender. The tip of the spleen was questionably palpable. There was noedemaof the legs. Neurologic examination was within normal limits. The rectal examination was negative. Lab-oratory studies revealed the fol lowing: Hemo-globin 9 gm/100 ml, hematocrit 26%, white blood cell count 5 ,700/cmm, myelocyte 1, seg-mented neutrophils 60, bands 1, lymphocytes 20, eosinophils 7, basophils 1, monocytes 10. Pe-ripheral blood smear revealed marked rouleaux 19 Ma, Maeda, Waldmann and Rebuck formation with three polychromatic normoblasts and fourorthochromatic normoblasts per hundred white cells. Platelets were 99,000/cmm. The sodium 130 meq/litre, potassium 4.2 meq/litre, chloride 101 meq/litre, carbon dioxide 27 milli-mols/litre. The BUN 23 mg/100 ml, creatinine 1.9 mg/100 ml. The CPK 17 U/litre, LDH 189 U/litre, SCOT 21 U/litre, alkaline phosphatase 4.8 U/litre. The calcium 13.2 mg/100 ml and phosphorus 5.1 mg/100 ml. Serum protein elec-trophoresis revealed total protein 10.6 gm/100 ml, albumin 3.56 gm/100 ml, alphai globulin 0.4 gm/100 ml,alphaj globulin 0.36 gm/100 ml, beta globulin 5.52 gm/100 ml and gamma globulin 0.22 gm/100 ml (Figure 1). Micro-double immu-nodiffusion of serum' revealed IgM less than 1.0 mg%, IgA greater than 2,000 mg%, IgG 166 mg% and IgE less than 0.04 mg%. Immunochemical analysis of serum either Immunoelectrophoresis or micro-double immunodiffusion indicated re-semblance of IgA myeloma and IgA was identi-fied as lambda type\ Bone marrow aspiration and biopsy revealed 77.4% of myeloma cells, 70.2% of which were poorly differentiated plasma cells(Figure2). Meta-static bone survey showed findings consistent with diffuse myelomatous involvement of the skeleton. Urinary immunodiffusion on two occa-sions was negative for IgM, IgG 0.3 mg%, IgA 2.1 mg%; the lambda light chain was positive. Scans of liver and spleen revealed hepatosplenomegaly. The prothrombin time was 14.5 seconds with a control of 11 seconds. Haptoglobin was negative. G-6PD was normal. Antinuclear factor was nega-tive. Cardiopathy was suspected but a cardiac fluoroscopy was negative. Intravenous pyelogram showed a non func t i on ing left k idney and a nephrotomogram revealed multiple calcified structures in the vicinity of the left kidney suggest-ing calcified cystic lesions. Rectal biopsy was performed and was negative for amyloici. The patient was treated suppor t ive jy for his m i ld azotemia. The consultant cardiologist considered possible cardiomyopathy secondary to plasma cell infiltration or amyloidosis. Chemotherapy was initiated on April 12, 1973, using Cytoxan, D-5W, BCNU, alkeran and pred-nisone. The patient responded and appeared to be in remission. Bone marrow in October, 1973, revealed degenerating fatty particles, very few hematopoiet ic elements and no ev idence of tumor. However, metastatic survey on November 9,1973 (Figure 3a) suggested diffuse myelomatous involvement of the skeleton. Clinical remission continued until March, 1974, when bone marrow again revealed70% of myeloma cells. On July 22, 1974, he was again admitted to the hospital because of low platelet count, anemia and a two-week history of intermittent palpitation. He was admitted to the hospital two more times in Sep-tember and in November, 1974, for multiple blood transfusions because of anemia and throm-bocytopenia with intermittent epistaxis. Bone sur-vey showed progression of involvement (Figure 3b). He was last admitted on December 5, 1974, with symptoms of generalized weakness, chest pain, fever and chills. On admission, his blood Figure 1 Serum protein electrophoresis, a monoclonal spike at beta region identified immuno-electrophorectically as IgA globulin. pressure was 120/20 mm Hg, with a pulse rate of 144 per minute. The cervical and inguinal lymph nodes were enlarged. Breath sounds were de-creased bilaterally with bilateral basilar rales, A grade II to VI systolic ejection murmur was heard at the left sternal border. The abdomen was slightly tender to palpation, but no organomegaly was present. Blood culture revealed growth of Staphylococcus aureus. Urine culture was nega-tive. Chest x-ray revealed infiltrates in the right lower lung field. The hemoglobin was 7.9 gm/100 ml, the WBC was 8,000/cmm with 84% poly-morphonuclear leukocytes, 5% bands, 4% lym-phocytes, 5% monocytes, the BUN was 45 mg/100 ml, creatinine 2.5 mg/100 ml. A diag-nosis was made of pneumonia with septicemia and he was treated with antibiotics. However, he expired on December 6, 1974. Postmortem Findings At autopsy, the bone m a r r o w i nc l ud ing ribs, s te rnum, vertebrae and pelv ic bones showed mu l t i p le smal l mye lomatous n o d -ules. The ex t ramedu l la ry mye lomatos is was fa i r ly w i d e spread, i n v o l v i n g t h e l iver (Figure 4) , spleen and abdomina l l y m p h nodes. Seen mic roscop ica l l y , the t u m o r was c o m -posed o f sheets of c losely packed p lasma c e l l s , p r e d o m i n a t e l y i m m a t u r e in f o r m . Some abdomina l l y m p h nodes were c o m -p l e t e l y r e p l a c e d by t u m o r c e l l s (F igu res 5a,b,c) . Both lungs showed marked edema w h i c h can be ascr ibed to sept icemia. There was also patchy b r o n c h o p n e u m o n i a . The 20 Multiple Myeloma Figure 2 Bone marrow smear. Note two immature plasma cells with fine nuclear chromatin pattern. Leishman's stain; magnification, X 1,650. left kidney was cystic with only a remnant of kidney tissue, the left renal artery and ureter were hypoplastic. These findings were con-sistent w i t h dysplasia. No amylo id was found in the heart or other organs. Family Survey of Immunoglobulins We also had an opportunity to perform a study of serum immunoglobulin levels of five family members, including the patient's father, three brothers and one sister. His mother had died of stroke at age 54, and serum was not avai lable. Serum immu-nog lobu l in levels o f a l l avai lable fami ly members are summarized in Table I. No significant abnormality was noted in IgA levels except for this patient. Comment Multiple myeloma is primarily a disease of the older age group, the highest incidence being reported in the ages from 50 to 70. One of us reviewed nine cases of myeloma in the literature of the pediatric age group adding another case of his own.^ EHowever, eight of these cases were reported 20 years ago. The precisely recorded serum and urine protein studies were found in only one case studied. Postmortem examination was per-formed in f ive c a s e s , f o u r being de-scribed in detail. Since myeloma patients under 35 years of age are very exceptional,' we have described clinical manifestations, hospital course, serum and urine protein studies and postmortem examinations. All of these findings show rather classic manifesta-tions of this disease in this young patient. 21 Ma, Maeda, Waldmann and Rebuck f Figure 3b Roentgenogram of the spine, taken 1 year after the previous f i lm (Figure 3a), showing a mottled appearance wi th end plate deformities and col-lapse of L-2 vertebra. Figure 3a Roentgenogram of the spine, showing diffuse demineralization. 22 Multiple Myeloma Table I FAMILY STUDY OF IMMUNOGLOBULIN LEVELS IgM IgG IgA Normal jange Patient 30-120 < LO 600-1400 166 30-135 >2, 000 Father 87 1. 557 103 Brother 1 74 2,368 107 Brother II lit 1, 757 208 Brother III 70 1, 525 210 Sister 67 1, 550 180 Myeloma IgA was identified as lambda type 23 Ma, Maeda, Waldmann and Rebuck Figure 4 Liver, diffusely infi l trated by myeloma cells. Hematoxylin and eosin; magnification, X150. Figure 5a Myelomatous infi l tration of the lymph node. Hematoxylin and eosin. Complete distortion of the normal architecture, magnification, X 90. 24 Multiple Myeloma Figure 5b Myelomatous infi l tration of the lymph node. Hematoxylin and eosin. Capsular inf i l trat ion, magnification, X175. Figure 5c Myelomatous infi l tration of the lymph node. Hematoxylin and eosin. Myeloma cells, magnification, X 1,650. 25 Ma, Maeda, Waldmann and Rebuck References 1. Hayashi H, LoGrippo GA, Perry MB, Mueller j M : Multiple myeloma proteins: Evaluation of laboratory methods as aids for diagnosis. Im-munological Communications 1(2):n3-121, 1972 2. Maeda K, Abesamis CM, Kuhn ML, Hyun BH: Multiple myeloma in childhood. Report of a case with breast tumor as a presenting man-ifestation. Amer / C//n Pathol 60:552-558, 1973 3. Berkheiser Ej: Multiple myeloma of children. Arch Surg 8:853-881, 1924 4. Jacoby P: Myelomatosis in a child of 8 years. Acta Radiologica 11:224-225, 1930 5. Rubinstein MA: Multiple myeloma as a form of leukemia. Blood 4:1049-1067, 1949 6. Slavens JJ: Multiple myeloma in a child. Amer/ Dis Child 47:821-835, 1934 7. Snapper I, Kahn A: Myelomatosis. Baltimore, Univers i ty Park Press, 1971, pp 113-116, 271-275 26 

Multiple myeloma in a 23-year-old man: A case report

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