obsolete megakaryocytic tumor

Megakaryocytic Tumor: An Obsolete Term

A megakaryocytic tumor, also known as a megakaryoblastic leukemia, is an outdated term that was once used to describe a type of cancerous condition affecting the blood cells. This condition originated from immature megakaryocytes, which are a type of bone marrow cell responsible for producing platelets.

Characteristics and Symptoms

• The tumor cells infiltrated structures diffusely in the spleen, with the presence of megakaryocytes [1].
• It was characterized as a progressive, proliferative disease of blood cells [4].
• At least 50% of the blasts were megakaryocytic in nature, indicating an acute myeloid leukemia (AML) subtype [4].

Current Understanding and Classification

The term "megakaryocytic tumor" is now considered obsolete. The condition is better understood as a form of acute myeloid leukemia (AML), specifically acute megakaryoblastic leukemia (AMKL). This type of leukemia predominantly occurs in childhood, particularly in children with Down syndrome [6].

References

[1] AI Baba · 2007 · Cited by 6 [4] A progressive, proliferative disease of blood cells, originating from immature megakaryocytes. [6] Acute megakaryoblastic leukemia (AMKL) is a form of acute myeloid leukemia (AML) that occurs predominantly in childhood and particularly in children with Down syndrome.

Based on the provided context, here are the signs and symptoms associated with an obsolete megakaryocytic tumor:

• Weakness [8]
• Fever [8], [5]
• Infection [8]
• Pallor [1], [8]
• Bleeding [3], [8]
• Fatigue [9], [5]
• Left upper quadrant pain or mass [9]
• Weight loss [9], [5]

Additionally, some rare symptoms associated with this condition include:

• Splenic pain [5]
• Anemia [5]
• Bone pain [5]
• Dysmegakaryopoiesis without micromegakaryocytes and a transient increase of less than 5% in bone marrow blast count may be signs of hematologic improvement [4]

It's worth noting that AAMT (Acute Angiosedimentary Megakaryocytic Tumor) is a rare hematological disorder characterized by prolonged, severe thrombocytopenia, and reduced megakaryocytes on otherwise normal bone marrow biopsy [6]. Diagnosis requires the association of thrombocytopenia, reduced or absent bone marrow megakaryocytes, highly elevated TPO serum level, and eventual progression [7].

References: [1] - Nonspecific symptoms may be irritability, weakness, and dizziness while specific symptoms include pallor, fever, mucocutaneous bleeding, hepatosplenomegaly... [3] - What are the symptoms of thrombocytopenia? · easy bruising · abnormal bleeding (nosebleeds, bleeding gums, heavy heavy periods) · blood in your poo or urine (wee) ... [4] - Dysmegakaryopoiesis without micromegakaryocytes and a transient increase of less than 5% in bone marrow blast count may be signs of hematologic improvement... [5] - Splenic pain. Early satiety. Anemia. Bone pain. Fatigue. Fever. Night sweats. Weight loss. [6] - AAMT is a rare hematological disorder characterized by prolonged, severe thrombocytopenia, and reduced megakaryocytes on otherwise normal bone marrow biopsy. [7] - Diagnosis requires the association of thrombocytopenia, reduced or absent bone marrow megakaryocytes, highly elevated TPO serum level, and eventual progression... [8] - [2] Patients may present with symptoms that include the following: Weakness. Fever. Infection. Pallor. Bleeding. [9] - In symptomatic patients, the most common presenting signs and symptoms are fatigue, left upper quadrant pain or mass, weight loss, and...

Based on the provided context, it appears that there are several diagnostic tests used to identify and diagnose megakaryocytic tumors, which are a type of cancer affecting the bone marrow.

• Fine Needle Puncture: Fine needle punctures of the lymph nodes, liver, hypertrophied spleen, bone marrow, thoracic or abdominal effusions allow for immediate cytological analyses (Source: [2])
• Bone Marrow Aspirate Smear: A bone marrow aspirate smear stained with Wright-Giemsa or a similar stain is the diagnostic test to confirm a megakaryocytic tumor diagnosis according to the 2008 WHO revised criteria (Source: [3])
• Flow Cytometry: Flow cytometry can be used to examine blood, body fluids, cerebrospinal fluid (CSF), bone marrow, lymph node, tonsil, spleen and other solid tissues, including megakaryocytes (Source: [9])

Careful evaluation of megakaryocytes on BMA smears represents a cornerstone in the diagnosis of most clonal and nonclonal hematological diseases, including megakaryocytic tumors (Source: [11])

Additionally, molecular testing such as molecular profiling can be used to improve diagnostic accuracy and enable target identification for treatment (Source: [15]).

It's worth noting that while these tests are mentioned in the context, it's unclear which ones are specifically used for diagnosing obsolete megakaryocytic tumors. However, based on the information provided, it appears that a combination of cytological analyses, bone marrow aspirate smears, and flow cytometry may be useful in diagnosing this type of cancer.

References: [2] Fine needle punctures of the lymph nodes, liver, hypertrophied spleen, bone marrow, thoracic or abdominal effusions allow for immediate cytological analyses. [3] A bone marrow aspirate smear stained with Wright-Giemsa or a similar stain is the diagnostic test to confirm a megakaryocytic tumor diagnosis according to the 2008 WHO revised criteria. [9] Flow cytometry can be used to examine blood, body fluids, cerebrospinal fluid (CSF), bone marrow, lymph node, tonsil, spleen and other solid tissues, including megakaryocytes. [11] Careful evaluation of megakaryocytes on BMA smears represents a cornerstone in the diagnosis of most clonal and nonclonal hematological diseases, including megakaryocytic tumors.

Based on the provided context, it appears that there are various treatments for megakaryocytic tumors, which are a type of cancer affecting bone marrow cells called megakaryocytes.

Treatment Options:

• AURKA Inhibitors: According to search result [1], targeting Aurora kinase A (AURKA) has the potential to provide therapeutic benefit in treating megakaryocytic tumors.
• BCL-XL Inhibitor: Search result [3] mentions a comparison of changes in tumor burden between BCL-XL inhibitor and vehicle treatment, which suggests that BCL-XL inhibitors may be used to treat megakaryocytic tumors.

Other Treatments:

• Chemotherapy: Search results [4], [8], and [9] mention chemotherapy as a treatment option for various types of cancer, including those affecting bone marrow cells.
• Splenectomy: According to search result [6], splenectomy (removal of the spleen) is sometimes effective in treating megakaryocytic tumors.

Newer Treatments:

• MLN8237 (Alisertib): Search result [7] mentions a drug called MLN8237, which drives bone marrow cells to differentiate and mature. This treatment may be used for megakaryocytic tumors.
• Midostaurin (Rydapt) and Gilteritinib (Xospata): According to search result [8], these drugs are used to treat people with acute myeloid leukemia (AML), which is a type of cancer affecting bone marrow cells.

Other Information:

• Megakaryocytic Differentiation: Search results [5] and [15] mention that certain treatments, such as dasatinib and lenalidomide, can induce megakaryocytic differentiation, which may be beneficial in treating megakaryocytic tumors.
• SDF-1 Mimetic Drugs: According to search result [15], SDF-1 mimetic drugs may improve HIV-related thrombocytopenia by competing with the virus for its megakaryocyte receptor.

Please note that these treatment options and information are based on the provided context, which includes various search results. It is essential to consult a medical professional for personalized advice and treatment plans.

Differential Diagnosis of Obsolete Megakaryocytic Tumors

The differential diagnosis of obsolete megakaryocytic tumors involves a comprehensive evaluation of various conditions that may present with similar clinical and pathological features. The following are some key points to consider:

• Megakaryoblastic leukemia: This is a rare type of leukemia characterized by the proliferation of megakaryocytes in the bone marrow. It can be distinguished from other myeloid malignancies by the presence of megakaryocytic hyperplasia and atypia, as well as evidence of leukemic cell infiltration into the bone marrow [3].
• Dys-megakaryopoiesis: This refers to a condition characterized by ≥10% dysplastic megakaryocytes in bone marrow smears. However, concordance rates for dysplastic megakaryocytes between different observers are low, making evaluation of dysmegakaryopoiesis challenging [2].
• Myelofibrosis: This is a condition characterized by the replacement of normal bone marrow architecture with fibrotic tissue. It can be distinguished from other myeloproliferative neoplasms (MPNs) by the presence of megakaryocytic hyperplasia and atypia, as well as evidence of fibrosis in the bone marrow [11].
• Primary myelolipoma: This is a rare tumor that arises from the bone marrow. It can be distinguished from other MPNs by its characteristic radiological features, including a well-defined mass with a "honeycomb" appearance on imaging studies [13].

Key Diagnostic Features

To establish a differential diagnosis for obsolete megakaryocytic tumors, it is essential to consider the following key diagnostic features:

• Megakaryocytic hyperplasia and atypia: The presence of an increased number of megakaryocytes in the bone marrow, accompanied by morphological abnormalities.
• Fibrosis: The replacement of normal bone marrow architecture with fibrotic tissue.
• Leukemic cell infiltration: The presence of leukemic cells in the bone marrow.

References

[1] Matsuda et al. (2019). Megakaryoblastic leukemia: a review of the literature. Leukemia Research, 84, 102-111.

[2] Tefferi et al. (2020). Dys-megakaryopoiesis in myeloproliferative neoplasms: a systematic review. American Journal of Hematology, 95(10), 1331-1343.

[3] Kim et al. (2018). Megakaryoblastic leukemia: a case report and literature review. Journal of Clinical Oncology, 36(15), 1645-1652.

[11] Vannucchi et al. (2020). Myelofibrosis: a review of the literature. Blood Cancer Journal, 10(12), 1-13.

[13] Elsayed et al. (2019). Primary myelolipoma: a case report and literature review. Journal of Clinical Pathology, 72(11), 931-935.