PMID- 35676715
OWN - NLM
STAT- MEDLINE
DCOM- 20220610
LR  - 20220716
IS  - 1477-7819 (Electronic)
IS  - 1477-7819 (Linking)
VI  - 20
IP  - 1
DP  - 2022 Jun 8
TI  - Extramedullary hematopoiesis in an inguinal lymph node: an unusual presentation 
      of primary myelofibrosis.
PG  - 186
LID - 10.1186/s12957-022-02660-9 [doi]
LID - 186
AB  - 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.
CI  - (c) 2022. The Author(s).
FAU - Yaqoob, Nausheen
AU  - Yaqoob N
AD  - Department of Histopathology, Indus Hospital and Health Network, Karachi, 75190, 
      Pakistan.
FAU - Mansoor, Neelum
AU  - Mansoor N
AD  - Department of Hematology, Indus Hospital and Health Network, Karachi, 75190, 
      Pakistan. neelum.mansoor@tih.org.pk.
FAU - Naveed, Hania
AU  - Naveed H
AD  - Department of Histopathology, Indus Hospital and Health Network, Karachi, 75190, 
      Pakistan.
FAU - Jamal, Saba
AU  - Jamal S
AD  - Department of Histopathology, Indus Hospital and Health Network, Karachi, 75190, 
      Pakistan.
LA  - eng
PT  - Case Reports
PT  - Journal Article
DEP - 20220608
PL  - England
TA  - World J Surg Oncol
JT  - World journal of surgical oncology
JID - 101170544
SB  - IM
MH  - *Bone Marrow Neoplasms
MH  - *Hematopoiesis, Extramedullary
MH  - Humans
MH  - In Situ Hybridization, Fluorescence
MH  - Lymph Nodes/pathology/surgery
MH  - Male
MH  - *Primary Myelofibrosis/complications/diagnosis/pathology
PMC - PMC9178870
OTO - NOTNLM
OT  - Cytogenetics
OT  - Extramedullary hematopoiesis
OT  - Immunohistochemistry
OT  - Primary myelofibrosis
COIS- The authors declare that they have no competing interests.
EDAT- 2022/06/09 06:00
MHDA- 2022/06/11 06:00
PMCR- 2022/06/08
CRDT- 2022/06/08 23:42
PHST- 2022/04/07 00:00 [received]
PHST- 2022/05/28 00:00 [accepted]
PHST- 2022/06/08 23:42 [entrez]
PHST- 2022/06/09 06:00 [pubmed]
PHST- 2022/06/11 06:00 [medline]
PHST- 2022/06/08 00:00 [pmc-release]
AID - 10.1186/s12957-022-02660-9 [pii]
AID - 2660 [pii]
AID - 10.1186/s12957-022-02660-9 [doi]
PST - epublish
SO  - World J Surg Oncol. 2022 Jun 8;20(1):186. doi: 10.1186/s12957-022-02660-9.