Thought I'd put together a few trivia questions for those of you who want to test your hairy cell leukemia knowledge:
Questions:
1) What causes hairy cell leukemia?
2) Who discovered hairy cell leukemia?
3) In what year was hairy cell leukemia discovered?
4) Where was hairy cell leukemia discovered?
5) What is the formal name of hairy cell leukemia?
6) What protein is overexpressed on hairy cells and the main target of monoclonal antibody therapy?
7) What does the "CD" in "CD20" stand for?
8) Name the primary chemotherapies for hairy cell leukemia?
9) When is Pentostatin typically used in lieu of Cladribine?
10) What's the term for treatment with a monoclonal antibody (mAb)?
11) What is the primary mAb used to treat hairy cell?
12) What other surface proteins are also targeted in hairy cell?
13) Name a well known HCL immunotoxin?
14) Who is Ira Pastan?
15) What is the bacterial toxin used in HA22, and what's its origin?
16) What is the typical cause of fever after treatment with Cladribine (commonly misdiagnosed as "a mystery infection")?
17) What are the three modes of cell death mediated by Rituxan?
18) Who invented Cladribine?
19) Which is the more effective approach for administering Cladribine?
5 day x 2 hour IV
or 24x7 drip?
20) At what stage of cellular development is the malignant mutation of hairy cell believed to occur?
21) What is the median age of an HCL patient at diagnosis?
22) Is HCL more prevalent in men or women?
23) What type of cells are hairy cells from?
24) What is the average period of remission for HCL after Cladribine chemotherapy?
25) What percentage of patients receiving Cladribine have a complete remission, partial remission, and no response?
26) How rare is hairy cell leukemia?
27) What does MRD stand for?
28) Is there a cure for hairy cell leukemia?
29) The first case of patient/doctor genetic rights involved hairy cell leukemia and what university?
30) What's the life expectancy of the average hairy cell leukemia patient?
31) When should hairy cell leukemia be treated?
32) What is Hairy Cell Leukemia?
33) What is Bruton's Tyrosine Kinase (BTK)?
34) What Hairy Cell Leukemia (HCL) treatment options are available to multiply relapsed and refractory patients who don't respond to chemotherapy?
Answers:
1) Most cases of classic HCL have a BRAF V600E DNA mutation, caused by a photon from sunlight inducing an RNA translation error in a B-cell (a type of white blood cell) during replication. This mutation is also common to 50% of melanoma cases, but in that case, it affects a skin cell. The causes of other variants of HCL are unknown.
2) Bertha A. Bouroncle, MD
3) 1958
4) Ohio State University
5) Reticuloendotheliosis
6) CD20
7) "Cluster of Differentiation"
8) Cladribine and Pentostatin
9) When a patient's counts are so low or health is so weak that a less sudden drop in counts is needed; however, it is sometimes used if a patient doesn't respond to Cladribine. Pentostatin is administered over several weeks whereas Cladribine is administered in 5 or 7 days.
10) mAb therapy
11) Rituximab (aka: Rituxan)
12) CD25, CD22
13) HA22
14) Head of NCI laboratory of molecular biology and immunotoxin development
15) Pseudomonas Exotoxin from Pseudomonas aeruginosa
16) Tumor Lysis (hairy cells dying)
17) Apoptosis (cell suicide), antibody-dependent cell-mediated cytotoxicity (ADCC), complement-dependent cytotoxicity (CDC)
18) Dr. Dennis Carson
19) Both are considered equally effective
20) Later in the differentiation process at the level of the germinal center B-cells, likely in the process of differentiating to a Memory B-cell (per Basso, et al, p.62, col 1).
21) 52
22) Men, by a ratio of 4:1
23) B-cells, a type of lymphocyte.
24) Ten years.
25) 80%, 15% and 5% respectively.
26) HCL accounts for only 2% of diagnosed/reported cases of leukemia. However, it may be more prevelant since it can remain "in-check" in many people without ever being diagnosed.
27) Minimal Residual Disease.
28) Not yet, but there are hairy cell clinical trials trying to establish a cure curve.
29) UCLA (link to court case)
30) The average patient will have a normal life expectancy.
31) In general, HCL should be treated when blood counts indicate one of the following conditions:
Platelets (PLT) < 100 K/uL
Hemoglobin (HGB) < 10 g/dL
Absolute Neutrophils (ANC) < 1.0 K/uL
however, Dr. Michael Grever, in his paper "How I treat Hairy Cell Leukemia (Blood, 10/2009)" states the following:
Questions:
1) What causes hairy cell leukemia?
2) Who discovered hairy cell leukemia?
3) In what year was hairy cell leukemia discovered?
4) Where was hairy cell leukemia discovered?
5) What is the formal name of hairy cell leukemia?
6) What protein is overexpressed on hairy cells and the main target of monoclonal antibody therapy?
7) What does the "CD" in "CD20" stand for?
8) Name the primary chemotherapies for hairy cell leukemia?
9) When is Pentostatin typically used in lieu of Cladribine?
10) What's the term for treatment with a monoclonal antibody (mAb)?
11) What is the primary mAb used to treat hairy cell?
12) What other surface proteins are also targeted in hairy cell?
13) Name a well known HCL immunotoxin?
14) Who is Ira Pastan?
15) What is the bacterial toxin used in HA22, and what's its origin?
16) What is the typical cause of fever after treatment with Cladribine (commonly misdiagnosed as "a mystery infection")?
17) What are the three modes of cell death mediated by Rituxan?
18) Who invented Cladribine?
19) Which is the more effective approach for administering Cladribine?
5 day x 2 hour IV
or 24x7 drip?
20) At what stage of cellular development is the malignant mutation of hairy cell believed to occur?
21) What is the median age of an HCL patient at diagnosis?
22) Is HCL more prevalent in men or women?
23) What type of cells are hairy cells from?
24) What is the average period of remission for HCL after Cladribine chemotherapy?
25) What percentage of patients receiving Cladribine have a complete remission, partial remission, and no response?
26) How rare is hairy cell leukemia?
27) What does MRD stand for?
28) Is there a cure for hairy cell leukemia?
29) The first case of patient/doctor genetic rights involved hairy cell leukemia and what university?
30) What's the life expectancy of the average hairy cell leukemia patient?
31) When should hairy cell leukemia be treated?
32) What is Hairy Cell Leukemia?
33) What is Bruton's Tyrosine Kinase (BTK)?
34) What Hairy Cell Leukemia (HCL) treatment options are available to multiply relapsed and refractory patients who don't respond to chemotherapy?
Answers:
1) Most cases of classic HCL have a BRAF V600E DNA mutation, caused by a photon from sunlight inducing an RNA translation error in a B-cell (a type of white blood cell) during replication. This mutation is also common to 50% of melanoma cases, but in that case, it affects a skin cell. The causes of other variants of HCL are unknown.
2) Bertha A. Bouroncle, MD
3) 1958
4) Ohio State University
5) Reticuloendotheliosis
6) CD20
7) "Cluster of Differentiation"
8) Cladribine and Pentostatin
9) When a patient's counts are so low or health is so weak that a less sudden drop in counts is needed; however, it is sometimes used if a patient doesn't respond to Cladribine. Pentostatin is administered over several weeks whereas Cladribine is administered in 5 or 7 days.
10) mAb therapy
11) Rituximab (aka: Rituxan)
12) CD25, CD22
13) HA22
14) Head of NCI laboratory of molecular biology and immunotoxin development
15) Pseudomonas Exotoxin from Pseudomonas aeruginosa
16) Tumor Lysis (hairy cells dying)
17) Apoptosis (cell suicide), antibody-dependent cell-mediated cytotoxicity (ADCC), complement-dependent cytotoxicity (CDC)
18) Dr. Dennis Carson
19) Both are considered equally effective
20) Later in the differentiation process at the level of the germinal center B-cells, likely in the process of differentiating to a Memory B-cell (per Basso, et al, p.62, col 1).
21) 52
22) Men, by a ratio of 4:1
23) B-cells, a type of lymphocyte.
24) Ten years.
25) 80%, 15% and 5% respectively.
26) HCL accounts for only 2% of diagnosed/reported cases of leukemia. However, it may be more prevelant since it can remain "in-check" in many people without ever being diagnosed.
27) Minimal Residual Disease.
28) Not yet, but there are hairy cell clinical trials trying to establish a cure curve.
29) UCLA (link to court case)
30) The average patient will have a normal life expectancy.
31) In general, HCL should be treated when blood counts indicate one of the following conditions:
Platelets (PLT) < 100 K/uL
Hemoglobin (HGB) < 10 g/dL
Absolute Neutrophils (ANC) < 1.0 K/uL
however, Dr. Michael Grever, in his paper "How I treat Hairy Cell Leukemia (Blood, 10/2009)" states the following:
"Therefore, I recommend that therapy be initiated when a declining trajectory predicts that the patient will reach a platelet count less than 100,000/uL or an absolute granulocyte count consistently below 1,000/uL."
32) HCL is an uncommon, chronic, neoplastic (malignant) disorder of B lymphocytes (a type of white blood cell) that predominantly afflicts middle-aged men. The patient usually presents with pancytopenia (broad spectrum reduction in blood counts -- low platelets, low white blood cells, low red blood cells). In the U.S., there are 500 to 800 cases of HCL annually, representing just 2% of all leukemias. Although it is incurable, it is highly treatable, with an average remission of 10 years.
33) BTK is an enzyme that's critical to the maturation of B-cells. Inhibiting it, with drugs like Ibrutinib, has proven to be a well-tolerated and effective means of targeting a variety of leukemias and lymphomas. Clinical trials using Ibrutinib to target hairy cell leukemia in relapsed and refractory patients are now underway.
34) Multiply relapsed and refractory Hairy Cell Leukemia (HCL) patients should consider the moxetumomab (moxe) clinical trial at NIH. A majority of patients have achieved complete remissions and in many cases eliminated MRD.
32) HCL is an uncommon, chronic, neoplastic (malignant) disorder of B lymphocytes (a type of white blood cell) that predominantly afflicts middle-aged men. The patient usually presents with pancytopenia (broad spectrum reduction in blood counts -- low platelets, low white blood cells, low red blood cells). In the U.S., there are 500 to 800 cases of HCL annually, representing just 2% of all leukemias. Although it is incurable, it is highly treatable, with an average remission of 10 years.
33) BTK is an enzyme that's critical to the maturation of B-cells. Inhibiting it, with drugs like Ibrutinib, has proven to be a well-tolerated and effective means of targeting a variety of leukemias and lymphomas. Clinical trials using Ibrutinib to target hairy cell leukemia in relapsed and refractory patients are now underway.
34) Multiply relapsed and refractory Hairy Cell Leukemia (HCL) patients should consider the moxetumomab (moxe) clinical trial at NIH. A majority of patients have achieved complete remissions and in many cases eliminated MRD.
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