Extramedullary hematopoiesis in an inguinal lymph node: an unusual presentation of primary myelofibrosis.
World J Surg Oncol
; 20(1): 186, 2022 Jun 08.
Article
em En
| MEDLINE
| ID: mdl-35676715
ABSTRACT
BACKGROUND:
Extramedullary hematopoiesis (EMH) is a proliferation of hematopoietic tissue outside of the bone marrow medullary space. It is a pathophysiologic response, more often associated with either a benign reactive hematological disease or a myeloproliferative neoplasm (MPN). Identification of EMH in adults is always pathologic. It is highly unlikely for a myeloproliferative neoplasm to present with inguinal lymphadenopathy. An unusual and complex case can be precisely diagnosed via a multidisciplinary approach involving experts from various modalities of laboratory. In this regard, the present case highlights the importance of an integrated approach in establishing the diagnosis. CASE PRESENTATION We report a case of a 61-year-old male patient of primary myelofibrosis who presented with extramedullary hematopoiesis in an inguinal lymph node. The patient initially presented with generalized symptoms including anemia, fatigue, abdominal pain, and weight loss. On examination, massive splenomegaly. Chest X-ray revealed consolidation which was secondary to right-sided pleural effusion. Therefore, he was suspected to have a lung carcinoma. However, lymph node biopsy revealed extensive fibrosis, consequently effacing the nodal architecture. An abnormal blood picture raised the possibility of bone marrow infiltration. Extensive panel of markers is tested on lymph node and bone trephine. Cytogenetic studies with G-banding analysis and fluorescence in situ hybridization (FISH) played a significant role in deriving clinical decision. Translocations identified in conventional cytogenetic workup led to the diagnosis of primary myelofibrosis. The case is being reported due to unusual presentation of PMF.CONCLUSION:
In conclusion, it is a distinctive case of myeloproliferative disorder initially presented with extramedullary hematopoiesis and through multidisciplinary workup successfully diagnosed as primary myelofibrosis. Awareness of unique clinical presentations and integrated approach towards diagnosis is the key to such challenging cases.Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Hematopoese Extramedular
/
Neoplasias da Medula Óssea
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Mielofibrose Primária
Tipo de estudo:
Diagnostic_studies
/
Prognostic_studies
Limite:
Humans
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Male
Idioma:
En
Revista:
World J Surg Oncol
Ano de publicação:
2022
Tipo de documento:
Article