Overview
The diverse presentation of acute leukemia subtypes often makes specific disease subtypes difficult to recognize. Few, however, are as clinically challenging for the practicing hematologic malignancy physician to recognize as acute panmyelosis with myelofibrosis (APMF). APMF is a rare type of acute myeloid leukemia (AML) that manifests with a rapidly fibrotic bone marrow and progressive cytopenias. Early recognition is essential, as APMF can quickly turn fatal, a consequence of the disease’s ubiquitous pathologic features, aggressive clinical course, and poor response to traditional treatments. Pathologically this AML subtype is recognizable for its heavily fibrotic marrow, increased overall cellularity, immaturity of all cell-line lineages, abnormal megakaryocyte population, and lack of driver mutations. Clinical features include lack of spleen enlargement, bony pain, fevers and other features associated with systemic inflammation.
In this chapter, we focus on how the hematologist and pathologist alike can best identify and manage the disease in light of the clinical challenges that accompany its rapid and progressive course. We discuss the background of this unique and deadly disease and what led to the identification of this disease from pathologically similar entities. Morphology and recognition of characteristic disease features, including immunophenotypic findings, cytogenetics and morphology are discussed. The identification and distinguishing features of
This subsequently causes a drop in red blood cells, platelets and normal white blood cell production. As an acute form of leukemia, AML progresses rapidly and is typically fatal within weeks or months if left untreated (Nazki et al., 2012).
Acute Lymphoblastic Leukemia, is the disease that affects children the most and because of the abnormal cells that are immature white blood cells which cannot help the body fight infections cause children with the disease to often get infections and have fevers (National Cancer Institute, 2002, p. 1). The symptoms that the patient with ALL may have depend on the number of abnormal cells of the patient where exactly the cells collect. Children patients with ALL have low amounts of healthy red blood cells and platelets, which cause less oxygen to be carried through the body because of the lack of red blood cells. Patients at times may look pale, feel weak, and tired causing bleeding and bruising very easily because of their lack of enough platelets. This condition is called anemia. Anemia is very much common in patients with acute lymphoblastic leukemia. Fever, fatigue, bone or joint pain, tiny red spots under the skin called petechiae are a couple of symptoms that the disease ALL has. Headaches with, or without vomiting also may occur if patient happens to have abnormal cells collecting in the brain or spinal cord (National Cancer Institute, 2002 para. 2).
In March 2011, Madyn (18 months of age) was diagnosed with acute lymphoblastic leukemia, a form of cancer. Doctors at Valley Children’s Hospital in Madera County, California, immediately began treatment, and Chemotherapy.
DK is a 51-year old male patient presenting with Acute Myeloid Leukemia AML. Two weeks ago while vacationing in Ocean City with his family, DK visited the local hospital with the symptoms of swollen leg, difficulty walking, pain, and fever. Bloodwork was performed at the local clinic and revealed anemia and low cell counts indicating possible leukemia. Patient returned to Baltimore and was admitted to Johns Hopkins Hospital 1 week ago. His current symptoms are swelling of lower legs, fever, low blood Oxygen level, anemia.
The town of Woburn, MA was stricken with an epidemic of different types of leukemia, including acute lymphoblastic leukemia, which affected 8 families. The mere notion that such rare cases occurred within a half mile radius of each other was a call for attention. It is the plaintiff 's claim that these cases were the result of a toxic contamination of the town’s water supply by the defendant 's unintentional, improper disposal of the following chemical waste products: Trichloroethylene (TCE) and Perchlorethylene (PCE). The plaintiff accuses the defendant of the following charges: negligence of chemical procedures including, but not limited to, illegal dumping of TCE, as well as PCE, and wrongful death, resulting from a depraved indifference to human life. Given the pain and suffering the victims and their families have undergone due to the defendant’s gross negligence, the plaintiff asks for compensation for and acknowledgement of wrongdoing on the defendant 's part. The plaintiff also calls for an injunction; in order for the defendant to resume further factory work, the company must propose, and be approved of, new procedures that strictly follow the enforced regulations that ensure the safe disposal of all chemical waste.
Myelofibrosis is a bone marrow disorder that leads to a disruption of the body’s ability to produce normal healthy blood cells. A rare form of chronic leukemia, the disease often worsens over time and may cause some patients to develop more serious types of leukemia. Conversely, other people have myelofibrosis and go for years without noticing any symptoms.
Acute Panmyelosis with Myelofibrosis (APMF) is a rare type of acute myeloid leukemia that manifests with a rapidly fibrotic bone marrow and progressive cytopenias. APMF is usually fatal, a consequence of the disease’s ubiquitous pathologic and disease features, rapidly progressive course, and poor response to traditional treatments. The disease is pathologically recognized for its heavily fibrotic marrow, increased overall cellularity, immaturity of all cell line lineages, abnormal megakaryocyte population, and lack of driver mutations that likely contribute to the rapid and progressive disease course. In this chapter, we focus on how the hematologist and pathologist alike can best identify and manage this disease in light of these clinical challenges. We discuss the background of this unique and deadly disease and what led to the identification of this disease from pathologically similar entities. Morphology and recognition of characteristic disease features, including immunophenotypic findings, cytogenetics and morphology are discussed. The identification and distinguishing features of this disease are explored in the setting of a comprehensive differential diagnoses that may mimic this pathologic state. Finally, we address disease prognosis and suggestions on management in light of the limited studies available regarding management.
Forms of MPNs, including polycythaemia vera can evolve into these forms of myelofibrosis (Tonkin et al., 2012). But, with well-managed essential thrombocythaemia, the life expectancy of a patient is increased to 20 years after their diagnosis (Tonkin et al., 2012). The primary myelofibrosis is categorized by a proliferation of primarily granulocytic and megakaryocytic lineage cells with reductions in erythroid precursors, which cause problems in the white blood cell functions and normal clotting of blood (Geyer & Orazi, 2016). According to Ye, Chen, Zheng, Zhu, Fu, and Huang (2017), with one cycle of decitabine (a myelodysplastic treatment drug) the blasts in a patient’s bone marrow decreased to 0.5%. After ten cycles, the mutation in the JAK2 decreases from 60.63% (average patient) to 0.01% (Ye et al.,
In Boston, Massachusetts, small groups of families sued US companies due to the constant leukemia and health deaths, caused by the poisonous chemicals dumped in the water, which got flowed into the communities water supply. Anne Anderson discovered that her child had leukemia. Searching for the cause, its discovered that there's a cluster of children with leukemia. Anderson, as well as the other families affected by the water, evolve into a law suit against two large companies.
Acute lymphoblastic leukemia (ALL) is a fast-growing cancer of the blood and the soft tissue inside the bones (bone marrow).
Research states, childhood leukemia is a cancer of the white blood cells. It is the most common cancer within children, and it occurs when irregular white blood cells form in the bone marrow. These irregular white blood cells start to rapidly travel through the blood stream and crowd out healthy blood cells. When the white blood cells travel through the blood stream and crowd out healthy cells, it increases the risk for infection (“Childhood Leukemia”). While childhood leukemia can be very traumatic, there are still ways to help families cope with this cancer.
This absence predisposes the patient’s cells to growth factor independence and therefore transformation. Another aspect of the transformation of note is that the proliferation takes longer due to slower leukemogenesis. This gives this disease an indolent course, which makes it particularly difficult to detect. The patient in this case was put on 400mg of Imatinib daily. The CML responded well to the treatment, the patient’s blood and marrow studies normalized. However, after five months the patient developed a lymphoid blast crisis. Bone marrow studies showed 94% lymphoid blast
Acute Lymphocytic Leukemia is a scary disease. The term “acute” means the disease can quickly spread and progress quickly and can prove fatal in months if left untreated. “Lymphocytic” simply means that it develops from immature forms of lymphocytes. Also known as Acute Lymphoblastic Leukemia, or ALL for short, it is a type of cancer that starts from the early form of white blood cells called lymphocytes in the bone marrow. ALL is the most common form of cancer in children, though adults can get it as well. For children, treatment results in a good chance of a cure. Adults have a significantly smaller chance of a cure with ALL. ALL generally invades the blood very quickly and has the potential to spread to other body parts such as the spleen,
Acute Promyelocytic Leukemia is the development of blood clotting or bleeding difficulty. It is where the bone marrow contains too much of promyelocytes cells, and the bone marrow has no space for healthy cells which go out of the body by bleeding. Acute Promyelocytic Leukemia is not genetic it is a DNA mutation. Acute Promyelocytic Leukemia affects the chromosomes 15 and 17. Promyelocytes is a granulocyte precursor that belongs to the granulocytic series. It is intermediate between myeloblasts and myelocytes, and it embraces some undifferentiated cytoplasmic granules. Some signs and symptoms of Acute Promyelocytic Leukemia are bleeding excessively with any small cut or any cut, blood in the urine, paleness, poor appetite, heavy nosebleeds,
Cancer is a deadly disease of uncontrolled cell division and has the potential to invade or spread to other parts of the body and affects normal functioning of the vital organs. Nowadays cancer is considered as leading cause of human death in all over the world and its burden will continue to increase due to continuous growth and ageing of world’s population (Chandra, 2012). Leukemia is one of the major types of cancer, affecting significant segments of the human population, especially children and adolescents. Based on diagnosis the most commonly forms of leukemia are acute myeloid leukemia, acute lymphoblastic leukemia, chronic myeloid leukemia and chronic lymphocytic leukemia (Goswami et al., 2014). Previously, it has been well documented