scholarly journals Case Report: Multisystem Langerhans Cell Histiocytosis (LCH) and Myelodysplastic Syndrome (MDS) in a 13-Month-Old Female

2021 ◽  
Vol 31 (3) ◽  
pp. 181
Author(s):  
Dian Sukma Hanggara ◽  
Desyi Desyi

<p>Langerhans Cell Histiocytosis or LCH and Myelodysplastic syndrome or MDS in children are rare diseases. It is estimated to be 1 to 4 cases per 1 million population and less than 5% of hematologic cases. MDS in LCH can occur due to genetic predisposition, impaired cytokine production, or secondary to chemotherapy.  The article reported a patient case of a 13-month-old female who came to hospital with paleness since two weeks before admission. The patient also experienced skin redness, abdominal distention, and weight loss. From the physical examination, anemia, maculopapular rash, and hepatosplenomegaly were obtained. From the laboratory test, anemia of hypochromic anisopoikilocytosis, monocytosis, thrombocytopenia, and hypoalbuminemia were obtained. On examination of bone marrow aspiration, MDS with Refractory Cytopenia of Childhood type or RCC was obtained. Positive results confirmed the diagnosis of Langerhans Cell Histiocytosis on CD68 and S100 in histopathological examinations.</p>

2019 ◽  
Vol 61 (4) ◽  
pp. 419-421 ◽  
Author(s):  
Yukiko Oh ◽  
Tomomi Hayase ◽  
Takane Ito ◽  
Yuta Kawahara ◽  
Akira Morimoto

2017 ◽  
Vol 14 (2) ◽  
pp. 264-267 ◽  
Author(s):  
Ines Vielgut ◽  
Bernadette Liegl-Atzwanger ◽  
Gerhard Bratschitsch ◽  
Andreas Leithner ◽  
Roman Radl

2020 ◽  
Vol 156 (7) ◽  
pp. 817 ◽  
Author(s):  
Sharad Khurana ◽  
Jason C. Sluzevich ◽  
Rong He ◽  
Danielle K. Reimer ◽  
Mohamed A. Kharfan-Dabaja ◽  
...  

2006 ◽  
Vol 33 (2) ◽  
pp. 171-174 ◽  
Author(s):  
Steven D. Billings ◽  
Christine P. Hans ◽  
Brian L. Schapiro ◽  
Robert W. Martin ◽  
David Fivenson ◽  
...  

2021 ◽  
Vol 40 ◽  
Author(s):  
Anis Mzabi ◽  
Maissa Thabet ◽  
Taghrid Tlili ◽  
Hend Zorgati ◽  
Jihed Anoun ◽  
...  

2021 ◽  
Vol 2021 ◽  
pp. 1-5
Author(s):  
Johanne Marie Holst ◽  
Marie Beck Enemark ◽  
Trine Lindhardt Plesner ◽  
Martin Bjerregaard Pedersen ◽  
Maja Ludvigsen ◽  
...  

Langerhans cell histiocytosis (LCH) is an infrequent disease, characterized by oligoclonal proliferation of immature myeloid-derived cells. However, the exact pathogenesis remains unknown. In rare cases, LCH is present in patients with concomitant myeloid proliferative neoplasms. Here, we describe a 69-year-old male, who presented with a maculopapular rash covering truncus, face, and scalp. A cutaneous ulcerating lesion on the right cheek led to a biopsy showing LCH. Lesional cells were BRAFV600E and JAK2V617F mutated. A bone marrow aspirate showed no infiltration of Langerhans cells, but alterations consistent with primary myelofibrosis (PMF) and a polymerase chain reaction test were positive for JAK2V617F. Our case highlights an uncommon condition of two hematological malignancies present in the same patient. The identification of the BRAFV600E mutation supports previous findings of this mutation in LCH. Interestingly, a JAK2V617F mutation was found in both LCH and PMF cells, indicating a possible clonal relationship between the two malignancies.


1998 ◽  
Vol 138 (5) ◽  
pp. 909-910 ◽  
Author(s):  
Marzano ◽  
Gasparini ◽  
Grammatica ◽  
De Juli ◽  
Caputo

2000 ◽  
Vol 111 (1) ◽  
pp. 258-262 ◽  
Author(s):  
Susi Scappaticci ◽  
Cesare Danesino ◽  
Elena Rossi ◽  
Catherine Klersy ◽  
Gian Mario Fiori ◽  
...  

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