Blastic Plasmacytoid Dendritic Cell Neoplasm - Symptoms, Diagnosis, and Treatment

Introduction

Blastic plasmacytoid dendritic cell neoplasm, also called NK cell lymphoma, blastic NK lymphoma is a blood cancer that is segregated from other types of blood cancers. Previously it was misdiagnosed for other blood and skin cancers. Though the studies on managing blastic plasmacytoid dendritic cell neoplasm are advancing, treatment to completely eradicate the disease is still not identified. Moreover, its prognosis is poor due to its occurrence and the aggressive nature of cancer. Most of the men are affected as compared to females.

What Is Blastic Plasmacytoid Dendritic Cell Neoplasm?

Blastic plasmacytoid dendritic cell neoplasm is an aggressive blood cancer that is derived from cells called plasmacytoid dendritic cells, also known as plasmacytoid monocytes or type 1 interferon-producing cells. It can occur at any age, predominantly affecting older males.

What Are the Causes of Blastic Plasmacytoid Dendritic Cell Neoplasm?

The causes of blastic plasmacytoid dendritic cell neoplasm are still being researched.

• There is no association with any bacterial or viral pathogens.

• The presence of other malignancies and syndromes, such as myelodysplastic syndrome and myeloproliferative disorders, can pave the way for blastic plasmacytoid dendritic cell neoplasm.

What Are the Findings Seen in Blastic Plasmacytoid Dendritic Cell Neoplasm?

Clinical findings of blastic plasmacytoid dendritic cell neoplasm vary from most common skin lesions to the effect on functional organs. A few clinical findings of blastic plasmacytoid dendritic cell neoplasm are as follows:

• Skin lesions are seen that can or cannot involve the bone marrow. These skin lesions resemble nodules, reddish to purplish papules.

• Eruptive lesions and isolated lesions are also seen. Eruptive lesions are suggestive of aggressive neoplasm, while isolated lesions are less aggressive.

• Lesions are also rarely seen in the oral cavity.

• Spleen and lymph nodes in the affected regions are also involved. In addition, an increase in the size of the spleen (splenomegaly) is observed.

• Rarely cerebrospinal fluid involvement is also seen.

What Are the Diagnostic Methods for Blastic Plasmacytoid Dendritic Cell Neoplasm?

Diagnosis of this neoplasm is highly challenging as the cells which occur during this course resemble the cells present in other blood cancers as well.

Following are a few diagnostic methods to detect blastic plasmacytoid dendritic cell neoplasm.

• Clinical Findings - Proper medical history, the record of the previous occurrence of neoplasm, and the symptoms are considered to obtain a provisional diagnosis of cancer and also to advise a proper treatment plan.

• Immunohistochemistry - It is a diagnostic method that uses specific antibodies and checks for the presence of antigens responsible for cancer.

• Flow Cytometry - This method shows the presence of a specific antigen and the amount of antigen present. It has the specificity to detect certain antigens which cannot be detected by immunohistochemistry.

• Complete Blood Profile - Decrease in blood cell counts such as red blood cells and white blood cells. Thrombocytopenia (decrease in the platelet count) is also observed.

• Bone Marrow Aspiration - Most leukemias or blood cancers are diagnosed by this method, where the samples of bone marrow are collected and examined in the laboratory.

• Immunophenotyping - The presence of certain markers on cancer cells can be detected by the immunophenotyping test.

What Are the Treatment Modalities for Blastic Plasmacytoid Dendritic Cell Neoplasm?

Though a wide range of antibodies is used, blastic plasmacytoid dendritic cell neoplasm shows resistance to chemotherapies. As a result, treatment outcomes are generally poor and short-lived responses.

Intensive induction regimens are found to be more effective than conventional chemotherapies.

Following are a few of the treatment modalities that are used in treating blastic plasmacytoid dendritic cell neoplasm.

• Tagrazofusp - It is an anti-cancer drug used in treating patients suffering from blastic plasmacytoid dendritic cell neoplasm for two or more years. It is used to enhance the treatment outcome after a hematopoietic stem cell transplant. It is found to be effective and safe in treating adult patients with a previous history of the same neoplasm.

• HCT (Hematopoietic Stem Cell Transplant) - In patients whose bone marrow is not able to produce enough blood cells, intravenous HCT is done to help in the production of blood cells.

• Transplantation - There are two types of stem cell transplantation.

• Allogenic Stem Cell Transplantation - Transplantation of blood cells from a healthy donor to the patient.

• Autologous Stem Cell Transplantation - Autologous, meaning self, refers to transplanting stem cells from the same individual after necessary treatments.

Allogenic stem cell transplants chowed less recurrence rate as compared to autogenic stem cell transplantation.

• Myeloablative Conditioning Regimen -This regimen for treating neoplasms is to reduce the relapse rate of the condition. Generally used along with HCT, it also aims at helping the patient live a disease-free life.

• Hypomethylating Agents - When patients cannot take up high-dose chemotherapy, combination therapy with hypomethylating agents are used.

• Target Therapies - Certain therapies that treat cancer by acting on the specific target cancer cells and proteins come under target therapies.

• Immunotherapies - Immunotherapies help in improving the immune system of the body to fight against cancer.

What Is the Prognosis of Blastic Plasmacytoid Dendritic Cell Neoplasm?

The prognosis of blastic plasmacytoid dendritic cell neoplasm is ultimately poor, where the patient's efficacy of life is diminished, and most of them hardly survive for one year. The higher rate of relapse of the disease, which can occur within two months and needs to be treated again with higher combination therapy, deteriorates the clinical condition of the patient. The most common misdiagnoses include acute myeloid leukemia, lupus erythematosus, and melanoma.

Conclusion

Blastic plasmacytoid dendritic cell neoplasm is a rare and aggressively occurring type of blood cancer. It was previously treated the same way in which leukemia was treated. Due to the advancements in diagnosis and efficiency of medical technologies and expert doctors, the condition is diagnosed and treated. There are young adults in whom the treatment was successful and also older adults in whom relapse was seen. Lower-intensity chemotherapy regimens are used in elder patients to obtain better treatment outcomes. The clinical condition of the patient also plays a major role in determining the success rate of the treatment. Hence it is necessary to visit a physician as and when the symptoms start to occur.