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Acute panmyelosis with myelofibrosis

 

 
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Acute panmyelosis with myelofibrosis
 
 
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Acute panmyelosis with myelofibrosis (APMF) it is a poorly-defined disorder that arises as either a clonal disorder, or following toxic exposure to the bone marrow
Bone marrow

Bone marrow is the flexible biological tissue found in the hollow interior of bones. In adults, marrow in large bones produces new blood cells....
.

marrow biopsy shows abnormal megakaryocytes, macrocytic erythropoiesis, and defects in neutrophil production and fibrosis of the marrow (myelofibrosis).

Clinically patients present with reduction in the count of all blood cells (pancytopenia
Pancytopenia

Pancytopenia is a medicine condition in which there is a reduction in the number of red blood cell and white blood cells, as well as platelets....
), a very few blasts in the peripheral blood and no or little spleen
Spleen

The spleen is an organ found in all vertebrate animals. In humans, the spleen is located in the abdomen of the body, where it functions in the destruction of redundant red blood cells, and holds a reservoir of blood....
 enlargement (splenomegaly
Splenomegaly

Splenomegaly is an enlargement of the spleen, which usually lies in the left upper quadrant of the human abdomen. It is one of the four cardinal signs of hypersplenism, the other three being cytopenia, normal or hyperplastic bone marrow, and a response to splenectomy....
).

Cells are usually CD34
CD34

CD34 molecule is a cluster of differentiation molecule present on certain cells within the human body. It is a cell surface glycoprotein and functions as a cell-cell adhesion factor....
 positive.

an survival is about 9 months.

Autologous stem cell transplantation has been used in treatment.

roversy remains today whether this disorder is a subtype of acute myeloid leukemia
Acute myeloid leukemia

Acute myeloid leukemia , also known as acute myelogenous leukemia, is a cancer of the myeloid line of white blood cells, characterized by the rapid proliferation of abnormal cells which accumulate in the bone marrow and interfere with haematopoiesis....
 or myelodysplastic syndromes; however, it is currently classified as a form of AML.






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Encyclopedia


Acute panmyelosis with myelofibrosis (APMF) it is a poorly-defined disorder that arises as either a clonal disorder, or following toxic exposure to the bone marrow
Bone marrow

Bone marrow is the flexible biological tissue found in the hollow interior of bones. In adults, marrow in large bones produces new blood cells....
.

Presentation

Bone marrow biopsy shows abnormal megakaryocytes, macrocytic erythropoiesis, and defects in neutrophil production and fibrosis of the marrow (myelofibrosis).

Clinically patients present with reduction in the count of all blood cells (pancytopenia
Pancytopenia

Pancytopenia is a medicine condition in which there is a reduction in the number of red blood cell and white blood cells, as well as platelets....
), a very few blasts in the peripheral blood and no or little spleen
Spleen

The spleen is an organ found in all vertebrate animals. In humans, the spleen is located in the abdomen of the body, where it functions in the destruction of redundant red blood cells, and holds a reservoir of blood....
 enlargement (splenomegaly
Splenomegaly

Splenomegaly is an enlargement of the spleen, which usually lies in the left upper quadrant of the human abdomen. It is one of the four cardinal signs of hypersplenism, the other three being cytopenia, normal or hyperplastic bone marrow, and a response to splenectomy....
).

Cells are usually CD34
CD34

CD34 molecule is a cluster of differentiation molecule present on certain cells within the human body. It is a cell surface glycoprotein and functions as a cell-cell adhesion factor....
 positive.

Prognosis and treatment

Median survival is about 9 months.

Autologous stem cell transplantation has been used in treatment.

Terminology

Controversy remains today whether this disorder is a subtype of acute myeloid leukemia
Acute myeloid leukemia

Acute myeloid leukemia , also known as acute myelogenous leukemia, is a cancer of the myeloid line of white blood cells, characterized by the rapid proliferation of abnormal cells which accumulate in the bone marrow and interfere with haematopoiesis....
 or myelodysplastic syndromes; however, it is currently classified as a form of AML.

See also

  • Acute myeloid leukemia
    Acute myeloid leukemia

    Acute myeloid leukemia , also known as acute myelogenous leukemia, is a cancer of the myeloid line of white blood cells, characterized by the rapid proliferation of abnormal cells which accumulate in the bone marrow and interfere with haematopoiesis....
  • Panmyelosis
    Panmyelosis

    Panmyelosis refers to a form of myeloid metaplasia.It is part of the presentation in acute panmyelosis with myelofibrosis....
  • Myelofibrosis
    Myelofibrosis

    Myelofibrosis, also known as myeloid metaplasia, chronic idiopathic myelofibrosis, and primary myelofibrosis, is a disorder of the bone marrow, in which the marrow undergoes fibrosis - replacement by fibrous tissue ....