Thymoma associated with hypogammaglobulinaemia and pure red cell aplasia

doi:10.1016/0002-9343(66)90054-4

The American Journal of Medicine

Volume 41, Issue 6, December 1966, Pages 974-980

https://doi.org/10.1016/0002-9343(66)90054-4 Get rights and content

Abstract

A sixty-eight year old woman is described who presented with recurrent acute bronchitis and was found to have hypogammaglobulinaemia of the primary acquired form and anaemia due to pure red cell aplasia. An unusual feature was the presence of normal numbers of plasma cells in the bone marrow. A myocardial infarct developed subsequently and the patient died. A thymoma was found at autopsy. The association of hypogammaglobulinaemia with pure red cell aplasia and thymoma is discussed. The precise mechanism of this association is unknown but is probably some form of immunologic defect.

References (33)

R.D.A. Peterson et al.
The pathogenesis of immunologic deficiency diseases
Am. J. Med.
(1965)
R.A. Good et al.
Clinical investigation of patients with agammaglobulinemia and hypogammaglobulinemia
Pediat. Clin. North America
(1960)
O. Larsson
Thymoma and systemic lupus erythematosus in the same patient
Lancet
(1963)
D. Alarcón-Segovia et al.
Systemic lupus erythematosus following thymectomy for myasthenia gravis. Report of two cases
Lancet
(1963)
C.W.H. Havard et al.
Thymic tumour and erythroblastic aplasia: report of three cases and review of the syndrome
Brit. J. Haemat.
(1960)
S.B. Andersen et al.
Pure red cell anaemia and thymoma
Acta haemat.
(1963)
R.A. Good
Agammaglobulinemia—a provocative experiment of nature
Bull. Univ. Minn. Hosp. & Minn. M. Fdn.
(1954)
C.A. Martin et al.
Acquired hypogammaglobulinemia in an adult: report of a case with clinical and experimental studies
New England J. Med.
(1956)
A.J. Ramos
Presentation of a case with discussion by V. Loeb.
J.A.M.A.
(1956)
A.T. Lambie et al.

J. Gafni et al.

Idiopathic aquired agammaglobulinemia associated with thymoma: report of two cases and review of the literature

New England J. Med.

(1960)

S. Godfrey

Thymoma with hypogammaglobulinaemia in an identical twin

Brit. M. J.

(1964)

F.A. Wollheim et al.

Primary “acquired” hypogammaglobulinemia. Clinical and genetic aspects of 9 cases

Acta med. scandinav.

(1964)

R.F. Jacox et al.

Hypogammaglobulinemia with thymoma and probable pulmonary infection with cytomegalovirus

New England J. Med.

(1964)

M. Rubin et al.

Clinical disorders associated with thymic tumors

Arch. Int. Med.

(1964)

G. Gehrmann et al.

Thymom und Agammaglobulinämie

Deutsche med. Wchnschr.

(1965)

Cited by (17)

Haemophilus influenzae pneumonia and immunodeficiency in association with thymoma-A presentation of Good's Syndrome
2011, Revista Portuguesa de Pneumologia
Good's Syndrome is a rare cause of immunodeficiency associated with thymoma. Patients with this syndrome are prone to infections with encapsulated microorganisms. The diagnosis may be delayed for a considerable time period even after the thymectomy.
We describe the case of a 70-year-old woman with a background of thymectomy who presented with pneumonia and gram negative sepsis. Haemophilus influenzae was found in blood cultures. Moreover, there was evidence of impaired B and T cell immunity consistent with Good's Syndrome. She was commenced on immunoglobulin replacement following treatment of sepsis and remains well 18 months after the initial presentation.
This case illustrates the importance of considering Good's Syndrome in the context of pneumonia and immunodeficiency associated with encapsulated organisms such as Haemophilus influenzae. This clinical entity is associated with a significant mortality and should be considered as a cause of immunodeficiency even years after thymectomy.
A Síndrome de Good é uma causa rara da imunodeficiência associada ao timoma. Os pacientes com esta síndrome são propensos a infecções por microrganismos encapsulados. O diagnóstico pode ser atrasado por bastante tempo, mesmo após a timectomia.
Descrevemos o caso de uma mulher de 70 anos com antecedentes de timectomia, que apresentava pneumonia e sepsis por agente gram negativo. O Haemophilus influenzae foi isolado em hemoculturas. Além disso, evidência de alterações da imunidade celular B e T, consistente com a Síndrome de Good. A doente iniciou terapêutica de substituição com imunoglobulina seguida de tratamento da sepsis e continua bem 18 meses após a apresentação inicial.
Este caso ilustra a importância de considerar a Síndrome de Good no contexto da pneumonia e imunodeficiência associadas a organismos encapsulados, como Haemophilus influenzae. Esta entidade clínica está associada a uma mortalidade significativa e deve ser considerada como uma causa de imunodeficiência mesmo anos depois da timectomia.
Good's syndrome remains a mystery after 55 years: A systematic review of the scientific evidence
2010, Clinical Immunology
Citation Excerpt :
57 patients (44.5%) died during the observation periods included in the case reports. 34 patients (59.6%) died as a result of an infection [2,7,14,19,23,28,37,40,44–46,59,75,93,99,101,115,117,138,140,142,150,156,157,159,166,172,175,185,186], 15 patients (26.3%) died as a result of a known non-infectious cause [2,38,105,110,135,145,168,171,173,175,176,184,186,188] and in 8 cases (14.1%) the cause of death was unclear [1,103,119,123,141,174,187,190]. Non-surgical regression of thymoma following immunosuppressive therapy was noticed in one case [64].
Good syndrome (GS) is a rare association of thymoma and immunodeficiency first described more than 50 years ago. However, this syndrome still remains a mystery to clinicians. We systematically reviewed all the clinical, laboratory and immunologic findings from 152 patients with Good syndrome. The syndrome has a worldwide distribution and approximately half of the cases (47%) have been described in Europe. The diagnosis of thymoma preceded the diagnosis of hypogammaglobulinemia, infection, or diarrhea in 42% of patients whereas in 38% of patients the diagnoses were made almost simultaneously within 2 months of each other. We found significant mortality in patients with this syndrome (44.5%). Astute clinical acumen and increased awareness about the clinical and immunological profile of this syndrome may increase early recognition of this syndrome and prevent mortality. Further studies are needed to elucidate this clinical entity.
Thymoma associated with pure red cell aplasia and Kaposi's sarcoma
2009, Revue des Maladies Respiratoires
Le thymome est une tumeur rare représentant 20 % des tumeurs médiastinales. Il peut être associé à des manifestations parathymiques. La fréquence de survenue d’un deuxième cancer dans le suivi à long terme est élevée.
Nous rapportons l’observation d’une femme âgée de 59 ans, qui consultait pour toux sèche traînante depuis 2 mois. L’imagerie thoracique révélait la présence d’un processus tissulaire expansif médiastinal antérieur droit associé à une atteinte pleurale bilatérale avec des adénopathies médiastinales. La fibroscopie bronchique était normale. La biopsie scannoguidée était non contributive. Une médiastinoscopie diagnostique était réalisée. L’examen histologique confirmait le diagnostic de thymome.
La patiente était traitée par 4 cures de chimiothérapie avec régression partielle de la masse. L’évolution était marquée par la survenue d’une anémie érythroblastopénique nécessitant sa mise sous corticothérapie avec disparition de l’anémie, mais apparition de nodules bleuâtres au niveau des 2 membres inférieurs dont la biopsie confirmait le diagnostic de sarcome de Kaposi. La patiente était mise sous chimiothérapie à base de vinblastine sans amélioration des lésions cutanées.
Notre observation associe un thymome, une anémie érythroblastopénique et un sarcome de Kaposi. Des désordres immunologiques semblent être à l’origine de cette association.
W. Ketata, S. Fouzi, S. Msaad, S. Ben Amira, I. Yangui, A. Ayoub
Thymoma is a rare tumor which represents about 20% of mediastinal tumors. It is associated with several parathymic conditions. A second cancer frequently occurs during long term follow up.
We report the case of a 59 year-old woman who presented with a two month history of a dry cough. Thoracic imaging revealed a soft tissue mass in the anterior mediastinum with associated bilateral pleural effusion and mediastinal lymphadenopathy. Fibreoptic bronchoscopy was normal. CT guided-biopsy was not contributive. A diagnostic mediastinoscopy was carried out. Histological examination confirmed the diagnosis of thymoma. The patient was treated with 4 cycles of chemotherapy which led to a partial regression of the mass. The course was marked by the occurrence of a pure red cell aplasia requiring corticosteroid therapy which caused the disappearance of the anemia. However, bluish nodules appeared on the patients legs which when biopsied were found to represent Kaposi’s sarcoma. The patient was treated with chemotherapy (vinblastine) without any improvement of the cutaneous lesions.
Our case describes the association of a thymoma, pure red cell aplasia and Kaposi’s sarcoma. Immunologic disorders seem to be in the origin of this association.
Pure red cell aplasia and hypogammaglobulinemia in a patient with thymoma
2009, Journal of the Chinese Medical Association
Both pure red cell aplasia (PRCA) and hypogammaglobulinemia are rarer conditions than myasthenia gravis (MG) in thymoma patients. Several articles have discussed the relation between PRCA and thymoma or hypogammaglobulinemia and thymoma, and their proper treatments. Instances of both PRCA and hypogammaglobulinemia in a thymoma patient are few and reported sporadically in the literature. We discuss a 46-year-old woman with thymoma and simultaneous PRCA and hypogammaglobulinemia who achieved complete remission from PRCA after perioperative steroid administration and extended thymectomy, and review the literature.
Thymoma and immunodeficiency (Good syndrome): A report of 2 unusual cases and review of the literature
2007, Annals of Allergy, Asthma and Immunology
Citation Excerpt :
Our patient is of Eastern European origin and thus may have been predisposed to the classic form of the disease; however, it is important to recognize that his underlying immunodeficiency and subsequently poor immune defense mechanisms may have played a role in manifestation of disease as well. Autoimmune diseases, such as myasthenia gravis, diabetes mellitus, polymyositis, pure red cell aplasia, aplastic anemia, and hemolytic anemia, may occur in association with Good syndrome.4,23-27 This association suggests a potential role of autoantibodies or autoreactive cell-mediated immunity in the pathogenesis of hypogammaglobulinemia.
Good syndrome is a rare cause of combined B- and T-cell immunodeficiency that occurs in association with a thymoma. Patients affected with Good syndrome have increased susceptibility to bacterial, fungal, viral, and opportunistic infections.
To describe 2 unusual cases of infections in patients with Good syndrome and review the literature.
Case 1 describes a 51-year-old woman with Good syndrome who presented with a 10-day history of diarrhea, nausea, and fevers. During her hospitalization she became pancytopenic and underwent a bone marrow biopsy and evaluation of her peripheral blood smear. Case 2 describes an 89-year-old man with Good syndrome who presented with a nonhealing leg ulcer, which underwent biopsy. A literature search through MEDLINE was performed. Keywords included Good syndrome, thymoma, hypogammaglobulinemia, immunodeficiency, and infection.
The peripheral blood smear in patient 1 showed ring-formed parasites in red blood cells suggestive of babesiosis. She began treatment with azithromycin, atovaquone, and doxycycline and recovered completely. Patient 2 underwent a biopsy of the foot. Immunohistochemical staining was positive for human herpesvirus 8 consistent with Kaposi sarcoma.
The concomitant occurrence of immunodeficiency and thymoma is known as Good syndrome. In contrast to other humoral immune defects, patients with this syndrome can develop opportunistic infections, and the prognosis appears less favorable compared with X-linked agammaglobulinemia or common variable immunodeficiency. Immunological investigations, including T-cell subsets, B cells, and quantitative immunoglobulins, should be considered part of the routine diagnostic evaluation in patients with a thymoma and recurrent infections.
Case report. Pure red blood cell aplasia complicating chronic lymphatic leukemia
1983, American Journal of the Medical Sciences
While the occurrence of autoimmune disorders such as hemolytic anemia and thrombocytopenia in chronic lymphatic leukemia (CLL) is well recognized, pure red blood cell aplasia (PRCA) complicating CLL is uncommon and intriguing. This is a report of the clinical course of a patient with B cell CLL who developed PRCA unresponsive to immunosuppressive therapy responsive to subsequent treatment with oxymethalone resulting in complete remission of PRCA.

View all citing articles on Scopus

^☆: From the Departments of Medicine and Pathology, Western General Hospital, Edinburgh, Scotland.

^†: Present address: Respiratory Diseases Unit, Northern General Hospital, Edinburgh, Scotland.

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Case reportThymoma associated with hypogammaglobulinaemia and pure red cell aplasia☆

Abstract

Am. J. Med.

Pediat. Clin. North America

Lancet

Lancet

Thymic tumour and erythroblastic aplasia: report of three cases and review of the syndrome

Brit. J. Haemat.

Pure red cell anaemia and thymoma

Acta haemat.

Agammaglobulinemia—a provocative experiment of nature

Bull. Univ. Minn. Hosp. & Minn. M. Fdn.

Acquired hypogammaglobulinemia in an adult: report of a case with clinical and experimental studies

New England J. Med.

Presentation of a case with discussion by V. Loeb.

J.A.M.A.

Idiopathic aquired agammaglobulinemia associated with thymoma: report of two cases and review of the literature

New England J. Med.

Thymoma with hypogammaglobulinaemia in an identical twin

Brit. M. J.

Primary “acquired” hypogammaglobulinemia. Clinical and genetic aspects of 9 cases

Acta med. scandinav.

Hypogammaglobulinemia with thymoma and probable pulmonary infection with cytomegalovirus

New England J. Med.

Clinical disorders associated with thymic tumors

Arch. Int. Med.

Thymom und Agammaglobulinämie

Deutsche med. Wchnschr.

Case report
Thymoma associated with hypogammaglobulinaemia and pure red cell aplasia☆