Mast Cell Leukemia with Ascites and Multiple Organs Damage
Mast Cell Leukemia (MCL), a rare subtype of systemic mastocytosis is defined by bone marrow involvement as atypical and aleukemic mast cells, if more than 20% and less than 10% of peripheral WBCs are mast cells, respectively. We met a case of aleukemic MCL presenting with anemia and ascites for 2 ye...
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| Format: | Article |
| Language: | English |
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Iranian Society of Pathology
2019-08-01
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| Series: | Iranian Journal of Pathology |
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| Online Access: | https://ijp.iranpath.org/article_36287_6af5983e0aee20545411254744a70639.pdf |
| _version_ | 1819096843380850688 |
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| author | Elham Jafari Ali Hadipour Behjat Kalantari Khandani Firoozeh Abolhasani |
| author_facet | Elham Jafari Ali Hadipour Behjat Kalantari Khandani Firoozeh Abolhasani |
| author_sort | Elham Jafari |
| collection | DOAJ |
| description | Mast Cell Leukemia (MCL), a rare subtype of systemic mastocytosis is defined by bone marrow involvement as atypical and aleukemic mast cells, if more than 20% and less than 10% of peripheral WBCs are mast cells, respectively. We met a case of aleukemic MCL presenting with anemia and ascites for 2 years, referred for BM evaluation, suspicious of leukemia. Our findings included BM involvement by diffused aggregates of oval- and spindle-shaped atypical mast cells, lacking mature mast cells and other hematopoietic cells. The mast cells were absent in peripheral blood smear. Further assessments showed positive reaction of mast cells metachromatic granules with Tryptase, Giemsa and Toluidine blue stains, the expression of CD117/KIT and CD45 by immunohistochemistery, and elevated level of serum Tryptase. Radiologic investigations revealed generalized lymphadenopathy, and massive hepatosplenomegaly, followed by the cervical lymphadenectomy, and liver wedge biopsy. Suspicious peritoneal lesions were identified and underwent excisional biopsy. Microscopic evaluations showed lymph nodes and liver involvement by cancer cells and the same features in peritoneal seeding. Multiple organs damage progressed in few months and the patient died despite surgery and chemotherapy. In conclusion, we report an extremely rarecase of aleukemic MCL with multiple organs damage such as liver, peritoneum, spleen, gastrointestinal tract and BM, presenting by ascites. According to this case and previous parallel studies, we suggest some clinicopathologic features in favor of poor prognosis, including the presence of multiple organs damage, hepatomegaly, ascites, peritoneal seeding, the absence of mature mast cells and other hematopoietic cells in the BM, and elevated serum Tryptase level. |
| first_indexed | 2024-12-22T00:05:38Z |
| format | Article |
| id | doaj.art-f411c793d98a429fb9439e8cc4a732b9 |
| institution | Directory Open Access Journal |
| issn | 1735-5303 2345-3656 |
| language | English |
| last_indexed | 2024-12-22T00:05:38Z |
| publishDate | 2019-08-01 |
| publisher | Iranian Society of Pathology |
| record_format | Article |
| series | Iranian Journal of Pathology |
| spelling | doaj.art-f411c793d98a429fb9439e8cc4a732b92022-12-21T18:45:34ZengIranian Society of PathologyIranian Journal of Pathology1735-53032345-36562019-08-0114326526910.30699/ijp.2019.96187.194836287Mast Cell Leukemia with Ascites and Multiple Organs DamageElham Jafari0Ali Hadipour1Behjat Kalantari Khandani2Firoozeh Abolhasani3Pathology and Stem Cells Research Center, Dept. of Pathology, Kerman University of Medical Science, Kerman, IranPathology and Stem Cells Research Center, Dept. of Pathology, Kerman University of Medical Science, Kerman, IranHematology and Oncology Division, Dept. of Internal Medicine, Kerman University of Medical Science, Kerman, IranDepartment of General Surgery, School of Medicine, Kerman University of Medical Sciences, Kerman, IranMast Cell Leukemia (MCL), a rare subtype of systemic mastocytosis is defined by bone marrow involvement as atypical and aleukemic mast cells, if more than 20% and less than 10% of peripheral WBCs are mast cells, respectively. We met a case of aleukemic MCL presenting with anemia and ascites for 2 years, referred for BM evaluation, suspicious of leukemia. Our findings included BM involvement by diffused aggregates of oval- and spindle-shaped atypical mast cells, lacking mature mast cells and other hematopoietic cells. The mast cells were absent in peripheral blood smear. Further assessments showed positive reaction of mast cells metachromatic granules with Tryptase, Giemsa and Toluidine blue stains, the expression of CD117/KIT and CD45 by immunohistochemistery, and elevated level of serum Tryptase. Radiologic investigations revealed generalized lymphadenopathy, and massive hepatosplenomegaly, followed by the cervical lymphadenectomy, and liver wedge biopsy. Suspicious peritoneal lesions were identified and underwent excisional biopsy. Microscopic evaluations showed lymph nodes and liver involvement by cancer cells and the same features in peritoneal seeding. Multiple organs damage progressed in few months and the patient died despite surgery and chemotherapy. In conclusion, we report an extremely rarecase of aleukemic MCL with multiple organs damage such as liver, peritoneum, spleen, gastrointestinal tract and BM, presenting by ascites. According to this case and previous parallel studies, we suggest some clinicopathologic features in favor of poor prognosis, including the presence of multiple organs damage, hepatomegaly, ascites, peritoneal seeding, the absence of mature mast cells and other hematopoietic cells in the BM, and elevated serum Tryptase level.https://ijp.iranpath.org/article_36287_6af5983e0aee20545411254744a70639.pdfmast cell leukemiasystemic mastocytosisascitesmultiple organ damageprognostic factors |
| spellingShingle | Elham Jafari Ali Hadipour Behjat Kalantari Khandani Firoozeh Abolhasani Mast Cell Leukemia with Ascites and Multiple Organs Damage Iranian Journal of Pathology mast cell leukemia systemic mastocytosis ascites multiple organ damage prognostic factors |
| title | Mast Cell Leukemia with Ascites and Multiple Organs Damage |
| title_full | Mast Cell Leukemia with Ascites and Multiple Organs Damage |
| title_fullStr | Mast Cell Leukemia with Ascites and Multiple Organs Damage |
| title_full_unstemmed | Mast Cell Leukemia with Ascites and Multiple Organs Damage |
| title_short | Mast Cell Leukemia with Ascites and Multiple Organs Damage |
| title_sort | mast cell leukemia with ascites and multiple organs damage |
| topic | mast cell leukemia systemic mastocytosis ascites multiple organ damage prognostic factors |
| url | https://ijp.iranpath.org/article_36287_6af5983e0aee20545411254744a70639.pdf |
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