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Systemic mastocytosis - a diagnostic challenge.

Lladó AC, Mihon CE, Silva M, Galzerano A - Rev Bras Hematol Hemoter (2014)

Bottom Line: The histopathologic analysis is essential for definitive diagnosis but there is no curative treatment.This is a rare clinical case of aggressive systemic mastocytosis for which palliative treatment can improve survival and quality of life.A brief review of the literature about this pathology is also included.

View Article: PubMed Central - PubMed

Affiliation: Hospital Santo António dos Capuchos, Lisbon, Portugal; Centro Hospitalar de Lisboa Central, Lisbon, Portugal. Electronic address: ana.llado@sapo.pt.

No MeSH data available.


Related in: MedlinePlus

Positive staining for CD117 (magnification: 400×) identifying atypical mast cells with fusiform shape.
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fig0020: Positive staining for CD117 (magnification: 400×) identifying atypical mast cells with fusiform shape.

Mentions: The sequential control blood tests showed increasing levels of bicytopenia (hemoglobin 11.2 g/dL and platelet count 122 × 109/L), leukocytosis (19.40 x 109/L), with no formula inversion, and LDH (572 U/L). With strong suspicion of a hematological disorder, a blood smear, myelogram and bone marrow biopsy were performed but all were unrevealing. The immunological study was negative and Paget's disease and multiple myeloma were excluded. Finally, a percutaneous L1 biopsy was performed. This was essential for the definitive diagnosis because it documented a multifocal infiltration of atypical mast cells, characterized by spindle-shaped and hypogranular forms, representing 5% of cellularity and forming cellular aggregates of more than 15 cells. An immunohistochemistry study identified positive staining for CD117 and tryptase of these mast cells confirming the diagnosis of SM (Figure 3, Figure 4, Figure 5). These features associated with peripheral blood test abnormalities (bicytopenia), weight loss and presence of osteolytic lesions are sufficient C findings that allowed the classification of an aggressive form of SM.


Systemic mastocytosis - a diagnostic challenge.

Lladó AC, Mihon CE, Silva M, Galzerano A - Rev Bras Hematol Hemoter (2014)

Positive staining for CD117 (magnification: 400×) identifying atypical mast cells with fusiform shape.
© Copyright Policy - CC BY-NC-ND
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4109736&req=5

fig0020: Positive staining for CD117 (magnification: 400×) identifying atypical mast cells with fusiform shape.
Mentions: The sequential control blood tests showed increasing levels of bicytopenia (hemoglobin 11.2 g/dL and platelet count 122 × 109/L), leukocytosis (19.40 x 109/L), with no formula inversion, and LDH (572 U/L). With strong suspicion of a hematological disorder, a blood smear, myelogram and bone marrow biopsy were performed but all were unrevealing. The immunological study was negative and Paget's disease and multiple myeloma were excluded. Finally, a percutaneous L1 biopsy was performed. This was essential for the definitive diagnosis because it documented a multifocal infiltration of atypical mast cells, characterized by spindle-shaped and hypogranular forms, representing 5% of cellularity and forming cellular aggregates of more than 15 cells. An immunohistochemistry study identified positive staining for CD117 and tryptase of these mast cells confirming the diagnosis of SM (Figure 3, Figure 4, Figure 5). These features associated with peripheral blood test abnormalities (bicytopenia), weight loss and presence of osteolytic lesions are sufficient C findings that allowed the classification of an aggressive form of SM.

Bottom Line: The histopathologic analysis is essential for definitive diagnosis but there is no curative treatment.This is a rare clinical case of aggressive systemic mastocytosis for which palliative treatment can improve survival and quality of life.A brief review of the literature about this pathology is also included.

View Article: PubMed Central - PubMed

Affiliation: Hospital Santo António dos Capuchos, Lisbon, Portugal; Centro Hospitalar de Lisboa Central, Lisbon, Portugal. Electronic address: ana.llado@sapo.pt.

No MeSH data available.


Related in: MedlinePlus