J THORAC CARDIOVASC SURG 79:860-863, 1980 Mediastinal lymph node hyperplasia, hypergammaglobulinemia, and anemia An adolescent girl with retarded development, delayed puberty, and hypergammaglobulinemia had anemia unresponsive to iron therapy that was cured when a mass of mediastinal giant lymph node hyperplasia was removed surgically. Rapid growth and development of secondary sexual characteristics occurred after operation. Follow-up studies made 20 years after operation showed compensated increased erythrocyte hemolysis and increased serum IgM. When mediastinal giant lymph node hyperplasia with abundant plasma cells is seen in childhood, there may be refractory anemia and immune disturbances. Similar giant lymph node hvperplasia without plasma cells may be found in an asymptomatic child or adult. The pertinent features of the few symptomatic cases reported are reviewed. Herbert C. Maier, M.D., and Sheldon C. Sommers, M.D., New York, N. Y. Giant lymph node hyperplasia, sometimes called Castleman's disease, mediastinal lymph nodal hamar- toma, angiofollicular lymph node hyperplasia, and other names, has been reported over 200 times since it was recognized in 1954. 1 Most of these lymphoid masses have been found within the thorax. Whereas the vast majority of patients with such lymphoid masses were asymptomatic, a significant percentage of those under 20 years of age who had giant lymph node hyperplasia have had a refractory anemia, usually with immunolog- ic abnormalities. All these occurred in the less than 10% referred to pathologically as the plasma cell sub- type by Keller, Hochholzer, and Castleman." Such a patient with a 20 year follow-up is herewith reported. Case report A 14-year-old white schoolgirl had an intrathoracic mass discovered on routine chest roentgenogram in November, 1958. The mass occupied the right posterior mediastinum from the clavicular level to the origin of the sixth rib, mea- sured 7.5 by 5 em, was well demarcated, and was located adjacent to the trachea and esophagus above the right main bronchus (Figs. I and 2). She was prepubertal, underde- veloped compared to siblings, and had suffered hemorrhages after tonsillectomy and tooth extraction several years pre- viously. On examination there were nasal bleeding points and a From the Departments of Surgery and Pathology, Lenox Hill Hospi- tal, New York, N. Y. Received for publication Sept. 14, 1979. Accepted for publication Nov. 5, 1979. Address for reprints: Herbert C. Maier, M.D., 50 E. 77th St., New York, N. Y. 10021. fading petechial rash of the lower extremities considered a result of cheerleader jumping activity. Laboratory values were as follows: hematocrit 30.2%, hemoglobin only 6.5 gm/dl, sedimentation rate 121 rnm/hr, red cell count 3.82 million, leukocyte count 6,300/mm a with a normal differen- tial, reticulocytes I. 3%, and platelet count 442,0001 mm. 3 There were moderate hypochromia, anisocytosis, and a few microcytic erythrocytes. Red cell fragility test showed be- ginning hemolysis at 0.46%, complete at 0.22%; control 0.44% to 0.32%, demonstrating an increased resistance to hemolysis. Serum iron level was low, 70 fLg/dl (normal 100 to 150), and serum iron binding capacity was 250 gmldl (normal 100 to 200). Bleeding time was 3.4 minutes and clotting time, 5 mm. Prothrombin time was 19 seconds (con- trol 15), and prothrombin consumption was 23 seconds (nor- mal). There was no response to vitamin K except for erythematous wheals. Bone marrow aspiration showed ery- throid and myeloid hyperplasia. Serum albumin was 3.3 and globulin 5.8 gm/dl with sig- nificantly increased gamma globulin. ASLO titer was 2,500 units. Because of this, the increased sedimentation rate, mi- gratory polyarthritis, and a continued low-grade fever follow- ing a respiratory infection, the possibility of acute rheumatic fever was considered, and later a hypersensitivity reaction to a sulfonamide taken for sore throat. Skin tests with tubercu- lin, histoplasmin, coccidioidin, blastomycin, and Kveim antigen were negative. Scalene lymph node biopsy was negative. The anemia persisted despite parenteral iron therapy, with hemoglobin remaining about 7 gmldl, although the level of serum iron reached 800 fLg/dl. In the succeeding months the hemoglobin rose to 9.5 gmldl. Five months after the original study, in June, 1959, the mass was removed. It was located between the superior vena cava superiorly, azygos vein inferiorly, and the vertebrae posteriorly, and it extended upward almost to the thoracic inlet. Adjacent enlarged lymph nodes up to 2 ern in size also were removed. 860 0022-5223/80/060860+04$00.4010 © 1980 The C. V. Mosby Co.
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Mediastinal lymph node hyperplasia, hypergammaglobulinemia, and anemiaMediastinal lymph node hyperplasia, hypergammaglobulinemia, and anemia
An adolescent girl with retarded development, delayed puberty, and hypergammaglobulinemia had anemia unresponsive to iron therapy that was cured when a mass of mediastinal giant lymph node hyperplasia was removed surgically. Rapid growth and development of secondary sexual characteristics occurred after operation. Follow-up studies made 20 years after operation showed compensated increased erythrocyte hemolysis and increased serum IgM. When mediastinal giant lymph node hyperplasia with abundant plasma cells is seen in childhood, there may be refractory anemia and immune disturbances. Similar giant lymph node hvperplasia without plasma cells may be found in an asymptomatic child or adult. The pertinent features of the few symptomatic cases reported are reviewed.
Herbert C. Maier, M.D., and Sheldon C. Sommers, M.D., New York, N. Y.
Giant lymph node hyperplasia, sometimes called Castleman's disease, mediastinal lymph nodal hamar- toma, angiofollicular lymph node hyperplasia, and other names, has been reported over 200 times since it was recognized in 1954. 1 Most of these lymphoid masses have been found within the thorax. Whereas the vast majority of patients with such lymphoid masses were asymptomatic, a significant percentage of those under 20 years of age who had giant lymph node hyperplasia have had a refractory anemia, usually with immunolog- ic abnormalities. All these occurred in the less than 10% referred to pathologically as the plasma cell sub- type by Keller, Hochholzer, and Castleman." Such a patient with a 20 year follow-up is herewith reported.
Case report
A 14-year-old white schoolgirl had an intrathoracic mass discovered on routine chest roentgenogram in November, 1958. The mass occupied the right posterior mediastinum from the clavicular level to the origin of the sixth rib, mea- sured 7.5 by 5 em, was well demarcated, and was located adjacent to the trachea and esophagus above the right main bronchus (Figs. I and 2). She was prepubertal, underde- veloped compared to siblings, and had suffered hemorrhages after tonsillectomy and tooth extraction several years pre- viously.
On examination there were nasal bleeding points and a
From the Departments of Surgery and Pathology, Lenox Hill Hospi- tal, New York, N. Y.
Received for publication Sept. 14, 1979. Accepted for publication Nov. 5, 1979. Address for reprints: Herbert C. Maier, M.D., 50 E. 77th St.,
New York, N. Y. 10021.
fading petechial rash of the lower extremities considered a result of cheerleader jumping activity. Laboratory values were as follows: hematocrit 30.2%, hemoglobin only 6.5 gm/dl, sedimentation rate 121 rnm/hr, red cell count 3.82 million, leukocyte count 6,300/mma with a normal differen- tial, reticulocytes I.3%, and platelet count 442,0001 mm. 3
There were moderate hypochromia, anisocytosis, and a few microcytic erythrocytes. Red cell fragility test showed be- ginning hemolysis at 0.46%, complete at 0.22%; control 0.44% to 0.32%, demonstrating an increased resistance to hemolysis. Serum iron level was low, 70 fLg/dl (normal 100 to 150), and serum iron binding capacity was 250 gmldl (normal 100 to 200). Bleeding time was 3.4 minutes and clotting time, 5 mm. Prothrombin time was 19 seconds (con- trol 15), and prothrombin consumption was 23 seconds (nor- mal). There was no response to vitamin K except for erythematous wheals. Bone marrow aspiration showed ery- throid and myeloid hyperplasia.
Serum albumin was 3.3 and globulin 5.8 gm/dl with sig- nificantly increased gamma globulin. ASLO titer was 2,500 units. Because of this, the increased sedimentation rate, mi- gratory polyarthritis, and a continued low-grade fever follow- ing a respiratory infection, the possibility of acute rheumatic fever was considered, and later a hypersensitivity reaction to a sulfonamide taken for sore throat. Skin tests with tubercu- lin, histoplasmin, coccidioidin, blastomycin, and Kveim antigen were negative. Scalene lymph node biopsy was negative.
The anemia persisted despite parenteral iron therapy, with hemoglobin remaining about 7 gmldl, although the level of serum iron reached 800 fLg/dl. In the succeeding months the hemoglobin rose to 9.5 gmldl.
Five months after the original study, in June, 1959, the mass was removed. It was located between the superior vena cava superiorly, azygos vein inferiorly, and the vertebrae posteriorly, and it extended upward almost to the thoracic inlet. Adjacent enlarged lymph nodes up to 2 ern in size also were removed.
860 0022-5223/80/060860+04$00.4010 © 1980 The C. V. Mosby Co.
Volume 79
Number 6 June, 1980
Fig. 1. Chest roentgenogram shows right paratracheal mass of homogeneou s dens ity . Lung fields are clear.
The preoperati ve hemoglobin was 11.1 gm/dl and it rose to 14.3 gm/dl I day after the operation. No blood was adminis- tered. Following the operation there was a rapid increase in growth characteristics and onset of puberty with menstruation starting shortly after operation and continuing normally.
In December, 1961, at the time of a left radial fracture, the hemoglobin value was 13.8 gm/dl and the red cell count 5.0 million /mm," with a normal leukocyte count and differential. In June , 1963, at age 19, while a student nurse, she was seen because of sinusitis and otitis media . The hemoglobin value was 12.6 gm/dl , and the red cell count 4 .52 million /rum ." with a normal leukocyte count, and differential. Chest roent- genograms have shown no further abnormalities. The patient has remained in good health . In 1963 serum electrophoresis showed normal alpha globulin s, decreased beta and gamma globul ins, and increased albumin .
In August, 1979, she agreed to follow-up studies. Interim history included delivery of three children who had hip dys- plasia. She was in good health . Abnormal laboratory findings included a low leukocyte count, 4, IOO/mm3 , with neutrophils 36%, bands 14%, lymphocytes 32%, monocytes II %, and eosinophils 7% . There were 142 eosinophlls/mms-i-a normal value . Hematocrit value was slightly subnormal (36.2%) and reticulocytes were increased (2.2%). The red cell count, he- moglobin , hematocrit, and red cell indices were normal. Sedimentation rate was normal . Blood T lymphoc ytes were 71% and B lymphocytes 6%-normal values. Blood chemis- try findings includ ing serum iron, were within normal limits. In summary, the patient had mild leukopenia and compen- sated increased hemolysis . Serum electrophoresis showed increased alpha-I globulin, 6.7% (normal 1.8% to 5.8%). Other serum prote in values were normal . The IgM was in- creased , 350 mg/dl (normal 75 to 270); IgA, IgG, and IgD were normal.
Mediastinal lymph node hyperpla sia 86 I
Fig. 2. Right lateral roentgenogram shows mass in the su- perior and posterior mediastinal area .
Pathological examination. The mass was encapsulated , weighed 69 gm , and measured 7.5 by 6 by 4 ern, It was light brown and homogeneous on section, with pinpoint gray areas. Microscopically there were many follicles with germi- nal centers, surrounded by mature lymphocytes , with moder- ate numbers of plasma cells and hyalinized fibrous tissue (Fig . 3). The diagno sis was hyperplastic mediastinal lymph node s, now called giant lymph node hyperplasia .
Discussion
Hypergammaglobulinemia and anemia resistant to iron therapy characterized this patient and some others with mediastinal giant lymph node hyperplasia ." Addi- tional symptoms reported in this syndrome, which she had also, are fever, arthralgia, and increased sedimen- tation rate. 4 Excision of the lymphoid mass corrects the anemia, hypoferremia, and the other symptoms. The growth rate is restored to normal and the serum gamma globulin falls to normal.
Most instances of giant lymph node hyperplasia have been intrathoracic, and others have occured in the ab- domen, neck, vulva, and extremities . Many of the mediastinal masses have been located in the pulmonary hilar regions, and others were paratracheal, particularly right sided. Several located paravertebrally have simu- lated neurogenic tumors, with roentgenographic evi- dence of rib distortion ." Chest pain and cough have
862 Maier and Sommers The Journal of
Thoracic and Cardiovascular
Surgery
Fig. 3. Large lymph follicles with pale germinal centers and intervening cords of small dark lymphocytes comprise the mass. Inset shows abundant mature plasma cells mixed with lymphocytes. (Hematoxylin and eosin stain; x 150, inset x625.)
been noted in some cases." By roentgenography the mass is often spherical or slightly lobulated, and roentgenologically may simulate a bronchogenic cyst. The mass apparently grows slowly but may not be evi- dent on a roentgenogram taken a few years previously. Giant lymph node hyperplasia was once thought of as misplaced thymic hyperplasia or thymoma, and later as a lymph node hamartoma, but these beliefs have now been superseded.
Plasma cells are numerous in the giant lymph node hyperplasia that is associated with anemia and immu- nologic disturbances. Other cases, called the vascular- hyaline type because of prominent thick-walled small blood vessels and areas of hyalinized collagen, are simply space-occupying lesions." Since plasma cells normally produce gamma globulins and are derived from B type lymphocytes, their activities are presuma- bly responsible for the immunologic abnormalities ob- served.
In some cases serum immunoglobulins G, M, and A have all been increased." In others two components, IgG and IgM, or IgG and IgA, were increased." In addition, the cellular immunity studied in one case showed that lymphocyte transformation in vitro after stimulation by phytohemagglutinin or allogeneic cells was partly blocked by a serum factor. 8 The blocking factor was possibly an alpha-lor an alpha-2 globulin. Another case with an abdominal mass and anemia had increased serum alpha globulins. The intra-abdominal masses more often have been associated with hepato- splenomegaly than those located in the thorax."
Most instances of anemia and giant lymph node hy- perplasia have been prepubertal, and apparently there is a natural amelioration in some individuals after puber- ty. Anemia noted in childhood might no longer be pres- ent when the mass is removed during adulthood. Fatigue, however, may persist until its removal. The immunologic alteration responsible for the refractory
Volume 79
Number 6 June, 1980
anemia has not been identified. Iron may be stored in abundance in the bone marrow, or found in the lym- phoid mass, but it is not utilized for erythropoiesis, and iron administration is ineffective.
Combined prednisone, nitrogen mustard, and metho- trexate therapy of one case after biopsy did not shrink the mass. 8 Radiation therapy has proved unsatisfactory in two cases either to shrink the mass or improve the anemia, but it did result in amelioration of a persistent low-grade fever in another abdominal case which had been considered inoperable. 10 Surgical removal of giant lymph node hyperplasia promptly restores the red cell count to normal and the other symptoms and laboratory abnormalities regress. Blades!' reported a 16 year cure, and the present patient is well after 20 years.
REFERENCES
Rosai J, Levine GD: Tumors of the Thymus, Washington, 1976, Armed Forces Institute of Pathology, pp 213-219
2 Keller AR, Hochholzer L, Castleman B: Hyaline-vascular and plasma-cell types of giant lymph node hyperplasia of the mediastinum and other locations. Cancer 29:670-683, 1972
3 Lee SL, Rosner F, Rivero I, Feldman F, Hurwitz A: Refractory anemia with abnormal iron metabolism. Its remission after resection of hyperplastic mediastinal lymph nodes. N Engl J Med 272:761-766, 1965
Mediastinal lymph node hyperplasia 8 6 3
4 Miller JS, Miller JJ III: Benign giant lymph node hy- perplasia presenting as fever of unknown origin. J Pediatr 87:237-239, 1975
5 Culver GJ, Choi B-K: Benign lymphoid hyperplasia (Castleman's tumor) mimicking a posterior mediastinal neurogenic tumor. Chest 62:516-517, 1972
6 Harrison EG, Bernatz PE: Angiofollicular mediastinal lymph node hyperplasia resembling thymoma. Arch Pathol 75:284-292, 1963
7 Boxer LA, Boxer GJ, Flair RC, Engstrom PF, Brown GS: Angiomatous lymphoid hamartoma associated with chronic anemia, hypoferremia, and hypergammaglobu- Iinemia. J Pediatr 81:66-70, 1972
8 Ballow M, Park BH, Dupont B, Caldwell RR, Lonsdale D, Good RA: Benign giant lymphoid hyperplasia of the mediastinum with associated abnormalities of the immune system. J Pediatr 84:418-420, 1974
9 Neerhout RC, Larson W, Mansur P: Mesenteric lymphoid hamartoma associated with chronic hypoferremia, anemia, growth failure and hyperglobulinemia. N Engl J Med 280:922-925, 1969
10 Nordstrom DG, Tewfik HH, Latourette HB: Plasma cell giant lymph node hyperplasia responding to radiation therapy. AJR 130: 169-171, 1978
An adolescent girl with retarded development, delayed puberty, and hypergammaglobulinemia had anemia unresponsive to iron therapy that was cured when a mass of mediastinal giant lymph node hyperplasia was removed surgically. Rapid growth and development of secondary sexual characteristics occurred after operation. Follow-up studies made 20 years after operation showed compensated increased erythrocyte hemolysis and increased serum IgM. When mediastinal giant lymph node hyperplasia with abundant plasma cells is seen in childhood, there may be refractory anemia and immune disturbances. Similar giant lymph node hvperplasia without plasma cells may be found in an asymptomatic child or adult. The pertinent features of the few symptomatic cases reported are reviewed.
Herbert C. Maier, M.D., and Sheldon C. Sommers, M.D., New York, N. Y.
Giant lymph node hyperplasia, sometimes called Castleman's disease, mediastinal lymph nodal hamar- toma, angiofollicular lymph node hyperplasia, and other names, has been reported over 200 times since it was recognized in 1954. 1 Most of these lymphoid masses have been found within the thorax. Whereas the vast majority of patients with such lymphoid masses were asymptomatic, a significant percentage of those under 20 years of age who had giant lymph node hyperplasia have had a refractory anemia, usually with immunolog- ic abnormalities. All these occurred in the less than 10% referred to pathologically as the plasma cell sub- type by Keller, Hochholzer, and Castleman." Such a patient with a 20 year follow-up is herewith reported.
Case report
A 14-year-old white schoolgirl had an intrathoracic mass discovered on routine chest roentgenogram in November, 1958. The mass occupied the right posterior mediastinum from the clavicular level to the origin of the sixth rib, mea- sured 7.5 by 5 em, was well demarcated, and was located adjacent to the trachea and esophagus above the right main bronchus (Figs. I and 2). She was prepubertal, underde- veloped compared to siblings, and had suffered hemorrhages after tonsillectomy and tooth extraction several years pre- viously.
On examination there were nasal bleeding points and a
From the Departments of Surgery and Pathology, Lenox Hill Hospi- tal, New York, N. Y.
Received for publication Sept. 14, 1979. Accepted for publication Nov. 5, 1979. Address for reprints: Herbert C. Maier, M.D., 50 E. 77th St.,
New York, N. Y. 10021.
fading petechial rash of the lower extremities considered a result of cheerleader jumping activity. Laboratory values were as follows: hematocrit 30.2%, hemoglobin only 6.5 gm/dl, sedimentation rate 121 rnm/hr, red cell count 3.82 million, leukocyte count 6,300/mma with a normal differen- tial, reticulocytes I.3%, and platelet count 442,0001 mm. 3
There were moderate hypochromia, anisocytosis, and a few microcytic erythrocytes. Red cell fragility test showed be- ginning hemolysis at 0.46%, complete at 0.22%; control 0.44% to 0.32%, demonstrating an increased resistance to hemolysis. Serum iron level was low, 70 fLg/dl (normal 100 to 150), and serum iron binding capacity was 250 gmldl (normal 100 to 200). Bleeding time was 3.4 minutes and clotting time, 5 mm. Prothrombin time was 19 seconds (con- trol 15), and prothrombin consumption was 23 seconds (nor- mal). There was no response to vitamin K except for erythematous wheals. Bone marrow aspiration showed ery- throid and myeloid hyperplasia.
Serum albumin was 3.3 and globulin 5.8 gm/dl with sig- nificantly increased gamma globulin. ASLO titer was 2,500 units. Because of this, the increased sedimentation rate, mi- gratory polyarthritis, and a continued low-grade fever follow- ing a respiratory infection, the possibility of acute rheumatic fever was considered, and later a hypersensitivity reaction to a sulfonamide taken for sore throat. Skin tests with tubercu- lin, histoplasmin, coccidioidin, blastomycin, and Kveim antigen were negative. Scalene lymph node biopsy was negative.
The anemia persisted despite parenteral iron therapy, with hemoglobin remaining about 7 gmldl, although the level of serum iron reached 800 fLg/dl. In the succeeding months the hemoglobin rose to 9.5 gmldl.
Five months after the original study, in June, 1959, the mass was removed. It was located between the superior vena cava superiorly, azygos vein inferiorly, and the vertebrae posteriorly, and it extended upward almost to the thoracic inlet. Adjacent enlarged lymph nodes up to 2 ern in size also were removed.
860 0022-5223/80/060860+04$00.4010 © 1980 The C. V. Mosby Co.
Volume 79
Number 6 June, 1980
Fig. 1. Chest roentgenogram shows right paratracheal mass of homogeneou s dens ity . Lung fields are clear.
The preoperati ve hemoglobin was 11.1 gm/dl and it rose to 14.3 gm/dl I day after the operation. No blood was adminis- tered. Following the operation there was a rapid increase in growth characteristics and onset of puberty with menstruation starting shortly after operation and continuing normally.
In December, 1961, at the time of a left radial fracture, the hemoglobin value was 13.8 gm/dl and the red cell count 5.0 million /mm," with a normal leukocyte count and differential. In June , 1963, at age 19, while a student nurse, she was seen because of sinusitis and otitis media . The hemoglobin value was 12.6 gm/dl , and the red cell count 4 .52 million /rum ." with a normal leukocyte count, and differential. Chest roent- genograms have shown no further abnormalities. The patient has remained in good health . In 1963 serum electrophoresis showed normal alpha globulin s, decreased beta and gamma globul ins, and increased albumin .
In August, 1979, she agreed to follow-up studies. Interim history included delivery of three children who had hip dys- plasia. She was in good health . Abnormal laboratory findings included a low leukocyte count, 4, IOO/mm3 , with neutrophils 36%, bands 14%, lymphocytes 32%, monocytes II %, and eosinophils 7% . There were 142 eosinophlls/mms-i-a normal value . Hematocrit value was slightly subnormal (36.2%) and reticulocytes were increased (2.2%). The red cell count, he- moglobin , hematocrit, and red cell indices were normal. Sedimentation rate was normal . Blood T lymphoc ytes were 71% and B lymphocytes 6%-normal values. Blood chemis- try findings includ ing serum iron, were within normal limits. In summary, the patient had mild leukopenia and compen- sated increased hemolysis . Serum electrophoresis showed increased alpha-I globulin, 6.7% (normal 1.8% to 5.8%). Other serum prote in values were normal . The IgM was in- creased , 350 mg/dl (normal 75 to 270); IgA, IgG, and IgD were normal.
Mediastinal lymph node hyperpla sia 86 I
Fig. 2. Right lateral roentgenogram shows mass in the su- perior and posterior mediastinal area .
Pathological examination. The mass was encapsulated , weighed 69 gm , and measured 7.5 by 6 by 4 ern, It was light brown and homogeneous on section, with pinpoint gray areas. Microscopically there were many follicles with germi- nal centers, surrounded by mature lymphocytes , with moder- ate numbers of plasma cells and hyalinized fibrous tissue (Fig . 3). The diagno sis was hyperplastic mediastinal lymph node s, now called giant lymph node hyperplasia .
Discussion
Hypergammaglobulinemia and anemia resistant to iron therapy characterized this patient and some others with mediastinal giant lymph node hyperplasia ." Addi- tional symptoms reported in this syndrome, which she had also, are fever, arthralgia, and increased sedimen- tation rate. 4 Excision of the lymphoid mass corrects the anemia, hypoferremia, and the other symptoms. The growth rate is restored to normal and the serum gamma globulin falls to normal.
Most instances of giant lymph node hyperplasia have been intrathoracic, and others have occured in the ab- domen, neck, vulva, and extremities . Many of the mediastinal masses have been located in the pulmonary hilar regions, and others were paratracheal, particularly right sided. Several located paravertebrally have simu- lated neurogenic tumors, with roentgenographic evi- dence of rib distortion ." Chest pain and cough have
862 Maier and Sommers The Journal of
Thoracic and Cardiovascular
Surgery
Fig. 3. Large lymph follicles with pale germinal centers and intervening cords of small dark lymphocytes comprise the mass. Inset shows abundant mature plasma cells mixed with lymphocytes. (Hematoxylin and eosin stain; x 150, inset x625.)
been noted in some cases." By roentgenography the mass is often spherical or slightly lobulated, and roentgenologically may simulate a bronchogenic cyst. The mass apparently grows slowly but may not be evi- dent on a roentgenogram taken a few years previously. Giant lymph node hyperplasia was once thought of as misplaced thymic hyperplasia or thymoma, and later as a lymph node hamartoma, but these beliefs have now been superseded.
Plasma cells are numerous in the giant lymph node hyperplasia that is associated with anemia and immu- nologic disturbances. Other cases, called the vascular- hyaline type because of prominent thick-walled small blood vessels and areas of hyalinized collagen, are simply space-occupying lesions." Since plasma cells normally produce gamma globulins and are derived from B type lymphocytes, their activities are presuma- bly responsible for the immunologic abnormalities ob- served.
In some cases serum immunoglobulins G, M, and A have all been increased." In others two components, IgG and IgM, or IgG and IgA, were increased." In addition, the cellular immunity studied in one case showed that lymphocyte transformation in vitro after stimulation by phytohemagglutinin or allogeneic cells was partly blocked by a serum factor. 8 The blocking factor was possibly an alpha-lor an alpha-2 globulin. Another case with an abdominal mass and anemia had increased serum alpha globulins. The intra-abdominal masses more often have been associated with hepato- splenomegaly than those located in the thorax."
Most instances of anemia and giant lymph node hy- perplasia have been prepubertal, and apparently there is a natural amelioration in some individuals after puber- ty. Anemia noted in childhood might no longer be pres- ent when the mass is removed during adulthood. Fatigue, however, may persist until its removal. The immunologic alteration responsible for the refractory
Volume 79
Number 6 June, 1980
anemia has not been identified. Iron may be stored in abundance in the bone marrow, or found in the lym- phoid mass, but it is not utilized for erythropoiesis, and iron administration is ineffective.
Combined prednisone, nitrogen mustard, and metho- trexate therapy of one case after biopsy did not shrink the mass. 8 Radiation therapy has proved unsatisfactory in two cases either to shrink the mass or improve the anemia, but it did result in amelioration of a persistent low-grade fever in another abdominal case which had been considered inoperable. 10 Surgical removal of giant lymph node hyperplasia promptly restores the red cell count to normal and the other symptoms and laboratory abnormalities regress. Blades!' reported a 16 year cure, and the present patient is well after 20 years.
REFERENCES
Rosai J, Levine GD: Tumors of the Thymus, Washington, 1976, Armed Forces Institute of Pathology, pp 213-219
2 Keller AR, Hochholzer L, Castleman B: Hyaline-vascular and plasma-cell types of giant lymph node hyperplasia of the mediastinum and other locations. Cancer 29:670-683, 1972
3 Lee SL, Rosner F, Rivero I, Feldman F, Hurwitz A: Refractory anemia with abnormal iron metabolism. Its remission after resection of hyperplastic mediastinal lymph nodes. N Engl J Med 272:761-766, 1965
Mediastinal lymph node hyperplasia 8 6 3
4 Miller JS, Miller JJ III: Benign giant lymph node hy- perplasia presenting as fever of unknown origin. J Pediatr 87:237-239, 1975
5 Culver GJ, Choi B-K: Benign lymphoid hyperplasia (Castleman's tumor) mimicking a posterior mediastinal neurogenic tumor. Chest 62:516-517, 1972
6 Harrison EG, Bernatz PE: Angiofollicular mediastinal lymph node hyperplasia resembling thymoma. Arch Pathol 75:284-292, 1963
7 Boxer LA, Boxer GJ, Flair RC, Engstrom PF, Brown GS: Angiomatous lymphoid hamartoma associated with chronic anemia, hypoferremia, and hypergammaglobu- Iinemia. J Pediatr 81:66-70, 1972
8 Ballow M, Park BH, Dupont B, Caldwell RR, Lonsdale D, Good RA: Benign giant lymphoid hyperplasia of the mediastinum with associated abnormalities of the immune system. J Pediatr 84:418-420, 1974
9 Neerhout RC, Larson W, Mansur P: Mesenteric lymphoid hamartoma associated with chronic hypoferremia, anemia, growth failure and hyperglobulinemia. N Engl J Med 280:922-925, 1969
10 Nordstrom DG, Tewfik HH, Latourette HB: Plasma cell giant lymph node hyperplasia responding to radiation therapy. AJR 130: 169-171, 1978
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