Respiratory tract

Case 1007964

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Femme de 19 ans présentant une pneumonie récidivante, non résolutive.

A l'imagerie, atteinte pulmonaire nodulaire, parfois excavée. Pas de lésion médiastinale, ou de modification pathologique dans d'autre localisation.

Biopsie chirurgicale pulmonaire.

 

19 yo woman complaining of a recurrent, non resolving pneumonia.

Nodular lesions of the lung, partly excavated on X-Rays. No mediastinal changes or pathology in other location.

Surgical biopsy of the lung.

 
     
     
       
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  CD30+   CD15-  
     
  CD3-   CD20-  
     
  LCA-   EMA- ALK-  
 

Diagnostic proposé

 

 

 

 

 

 

 

Lymphome de Hodgkin, classique

 

Proposed diagnosis

 

 

 

 

 

 

 

Classic Hodgkin's lymphoma

 
  Arguments (in the text)- Reference      
 

Hodgkin's Lymphoma Presenting in the Lung
in
Dail and Hammar’s Pulmonary Pathology, 3rd.Ed
J. Tomashefski, JR and al.
Springer 2008

 
  Hodgkin's lymphoma (formerly Hodgkin's disease) is broadly categorized into the classic type containing neoplastic Hodgkin cells and Reed-Sternberg cells and the lymphocyte predominant type containing lymphocytic and histiocytic (L&H) Reed-Sternberg cell variants. The vast majority of Hodgkin's lymphomas involving the lung are of classical type and therefore the discussion focuses on this entity.  
  Classic Hodgkin's lymphoma is characterized by the presence of large mononucleated Hodgkin cells and multinucleated Reed-Sternberg cells in a polymorphous background containing variable proportions of small lymphocytes, plasma cells, histiocytes, and eosinophils. Hodgkin and Reed-Sternberg (HRS) cells lack most B-cell– and T-cell–associated antigens and rather express antigens not often expressed by lymphocytes such as CD15 and the activation-associated antigen CD30. For this reason the cellular origin of these cells was unclear. Detailed molecular genetic and immunophenotypic analysis have revealed that in most cases of classic Hodgkin's lymphoma (>95%) the HRS cells are derived from post-germinal center B cells, and in the remainder they are derived from T cells. Gene expression profiling data indicate that HRS cells are all closely related, however, regardless of their cellular origin. A unifying feature of HRS cells is their ability to "turn off" the expression of lineage-specific genes such as productively rearranged immunoglobulin genes in the B-cell type. The mechanism by which this occurs is unknown; however, in normal B cells this would be a lethal event inducing apoptosis (programmed cell death). The HRS cells, in contrast, do not undergo apoptosis in response to these abnormalities, possibly due to the expression of antiapoptotic proteins such as cFLIP and cIAP2 (reviewed by Diehl et al.).  
  Systemic classic Hodgkin's lymphoma (not the lymphocyte predominance type) frequently involves the lung or pleura. This is especially with relapsed systemic disease where the lung is involved in approximately 40% of the cases. In contrast, primary pulmonary Hodgkin's lymphoma is considered rare, and some have disputed its existence. Nevertheless, case reports and small series of primary pulmonary Hodgkin's lymphoma have appeared for many years and 61 cases in the literature were reviewed.  
  Primary pulmonary Hodgkin's lymphoma occurs more frequently in women (2:1), and patients are older than those with primary nodal disease; the average age is 33 years for men and 51 years for women. The majority were symptomatic, and the symptoms were (in decreasing order of frequency) cough, fever, weight loss, dyspnea, fatigue, anorexia, chest pain, and pruritis. Radiographically, reticulonodular infiltrates and single or multiple nodules are described. Cavitation is not uncommon. These findings are similar to those in systemic Hodgkin's lymphoma with pulmonary involvement, where poorly marginated nodules and parenchymal infiltrates have been described. Cavitation, however, is not often encountered in this setting. In Hodgkin's lymphoma, CT appears to be superior to standard radiology in both detecting and characterizing the pulmonary nodules.  
  The gross findings parallel the radiologic findings. The histologic findings of pulmonary Hodgkin's lymphoma are identical to those in lymph nodes: diagnostic Reed–Sternberg cells with large multilobate nuclei with inclusion-like macronucleoli are present in the appropriate polymorphous cellular milieu composed of varying proportions of small lymphocytes, eosinophils, plasma cells, and histiocytes. It is worthwhile to bear in mind that many cases of primary (and secondary) pulmonary Hodgkin's lymphoma are the nodular sclerosis subtype, and therefore the majority of the Reed-Sternberg cells are lacunar variants, and the classic bilobed Reed-Sternberg cells may be few in number. The diagnosis of Hodgkin's lymphoma is usually confirmed by demonstrating the appropriate immunophenotype (CD30 and CD 15 positive, CD45 negative) in the Reed-Sternberg cells and variants. In small nodules and diffuse infiltrates, a lymphatic distribution of infiltration can be discerned; vascular infiltration occurs. Other patterns include a pneumonic growth pattern (in which the infiltrate fills alveoli in a consolidative fashion), endobronchial lesions, and extensive subpleural or pleural involvement. Some cases may show a dramatic sarcoid-like granulomatous reaction.  
 

The patients in Yousem et al.'s series were treated with conventional chemotherapeutic protocols for Hodgkin's disease. Approximately half showed a favorable response to combination chemotherapy with long-term remissions. Unfavorable prognosis was linked to B-symptoms, age greater than 60 years, and bilateral disease.