HEMATOLOGIC/BLOOD CANCERS

Adult Acute Lymphoblastic Leukemia adult

Description: is a t ype of cancer in which leukemic l ymphoblasts invade the bone
marrow and the bone marrow starts making too many l ymphocytes. Previous
chemotherapy and radiation therapy may increase the risk of developing ALL. A
biopsy of the bone marrow can let the physician know about the degree of bone
marrow involvement. ALL in adult patients do not have a very good success r ate
and have a lower cure rate
(http://www.cancer. gov/cancertopics/pdq/treatment/adultALL/HealthProfessional ).
The disease is classified as leukemia rather than l ymphoma if there are >25% bone
marrow blasts with or without a mass.
Methods of treatment : 3 different ways: 1. Untreated ALL: induction therapy &
CNS prophal yxis therapy. 2. ALL in remission: similar to untreated ALL and 3.
Recurrent ALL: chemotherapy and palliative radiotherapy
(http://www.cancer. gov/cancertopics/pdq/treatment/adultALL/HealthProfessional/
page4). Main treatment in adults involves long term use of chemotherapy drugs.
Treatment to prevent leukemic cells from spreading to the CNS involves
intrathecal chemotherapy injecti on in which chemotherapy drugs like methotrexate
or prednisone or directly injected into the spinal fluid. Radiation therapy of brain
and spinal cord is also an option to treat CNS. Cranial irradiation is done for CNS
prophylaxis in high risk patients. Testicular irradiation is done to prevent rel apse
in the testes. In case of bone marrow transplantation, a total body irradiation is
performed. Radiation therapy is used sometimes to shrink the tumors that press on
the trachea and cause breathing problems. Radiation therapy can also be used in an
area of bone invaded by leukemic cells if chemotherapy did not help. In the 1960s,
the problem of CNS recurrence was addressed with CNS radiation and IT
chemotherapy. Subsequent improvements in ALL cure rates during t he last 2 to 3
decades has resulted from improved risk stratification of patients with more
effective, tailored multidrug regimens.
Conventional RT Dose/fx: 24-Gy cranialirradiation were recognized in the 1980s
and 1990s, leading to the elimination of cranial RT in favor of intermediate - or
high-dose MTX in all but high-risk patients or those with CNS-3 disease. In
addition, with the improvements in systemic therapy, testicular relapse in males
has become a rare event. Testicular irradiation is rarel y necessary except in the
setting of testicular relapse or bone marrow transplantation (Textbook of
Radiation Oncology, Phillips Liebel). TBI is used for immunosupression before
bone marrow transplant and killing cancer cells throughout the body. The total
dose to the TBI is limited to 12 to 14 Gy in 1.5 to 2 Gy/fx delivered 2 to 3 times a
day (Clinical Radiation Oncology by Gunderson).
Clinical Trials RT Dose: For adults with ALL, various protocols have used 24 Gy
and others employ 18 Gy. (Cortes J. Central nervous system involvement in adult
acute l ymphocytic leukemia. Hematol Oncol Clin North Am 2001;15:145162) In
order to reduce the toxicity of prophylactic cranial radiation, several
investigations have explored a reduction in radiation dose. The use of 18 Gy in 9
or 10 fractions along with IT MTX yields comparable disease control rates as 24
Gy. The Dana Farber Cancer Institute ALL Consortium has been studying the role
of hyperfractionated cranial radiation (0.9 Gy twice dail y) compared with standard
daily fractionated treatments (1.8 Gy daily) to 18 Gy in high-risk patients.
Preliminary results show excellent CNS control rates with both the standard or
hyperfractionated treatments (Perez and Bradys Textbook of Radiation Oncology)
Acute Lymphoblastic Leukemia, Children
Description: Childhood ALLs tend to respond better to treatments such as
chemotherapy. Childrens bodies also tend to tolerate chemotherapy better than
adults bodies do. But cancer treatments such as chemotherapy and radiation
therapy can have some long-term side effects, so children who have had cancer
will need careful attention for the rest of their lives. The cure rate for childhood
ALL is about 90%
(http://www.cancer. gov/cancertopics/pdq/treatment/childALL/HealthProfessional ).
Methods of Treatment: Treatment of childhood ALL t ypicall y involves
chemotherapy given for 2 to 3 years. Historicall y, as multiagent chemotherapy
proved to be highl y effective in producing remis sions in childhood ALL, numerous
investigators noted a significant increase in CNS relapses. The CNS was
recognized as a sanctuary site, protected from chemotherapy by the bloodbrain
barrier. In addition, CNS recurrences invariabl y led to systemic recurre nce,
suggesting that CNS disease was capable of reseeding the blood and marrow.
Cranial radiotherapy may be used to prevent CNS relapse of leukemia. However,
children at younger age who are irradiated to a big volume suffers from worse
neurocognitive funct ion. Central nervous system prophalaxysis dose of 24 Gy
whole brain irradiation with intrathecal chemotherapy showed 13 point IQ drop
(Clinical Radiation Oncology by Genderson)
RT Dose/fx: In current practice, cranial radiation is employed selectivel y in high-
risk ALL patients, with a general trend toward decreasing radiation dosage to
address toxicity concerns. Unless indicated otherwise in a specific treatment
protocol, the radiation prescription for prophylactic cranial radiation to prevent
ALL relapse is 18 Gy in 9 or 10 fractions ( Textbook of Radiation Oncology by
Phillips and Liebel).
Clinical Trials: in 19621967 from St. Judes Cancer Research Hospital,
established that CSI to 24 Gy in 15 to 16 fractions reduced the isolated CNS
relapse rate from 67% to 4%. The Childrens Cancer Study Group (CCSG) trial no.
101 compared: (a) 24-Gy CSI plus extended field RT encompassing the liver,
spleen, and gonads; (b) 24-Gy CSI alone; (c) 24-Gy cranial RT plus IT MTX; and
(d) IT MTX alone. Overall, the different radiation regimens were comparable in
preventing CNS relapse while statisticall y superior to chemotherapy alone. a dose
of 18 Gy for prophylactic cranial irradiation has become more or l ess standard for
pediatric ALL (Pediatric leukemia and lymphomas In: Gunderson LL, Tepper TE
(eds) Clinical radiation oncology, 2nd edn. Churchill Livingstone, Philadelphia) .
The pediatric blood and marrow transplant consortium conducted a study to
compare bulsufan or TBI in children with ALL. The study showed that there was a
greater probabilit y to event free survival for the patients that received 18Gy of
TBI to the central nervous system before receiving bulsufan (Clinical Radiation
Oncology, Pg 356).
Acute Myeloid Leukemia, adult
Description: Adult acute myeloid leukemia (AML) is a t ype of cancer in which the
bone marrow makes abnormal myeloblasts (a t ype of white blood cell) , red blood
cells, or platelets
(http://www.cancer. gov/cancertopics/pdq/treatment/adultAML/Patient/page1/AllPa
ges/Print)
Methods of Tx: Chemotherapy, Radiation therapy and stem cell transplant.
Conventional RT Dose: TBI is performed as part of a bone marrow transplant. The
radiotherapy kills off all bone marrow, including the leukemia cells. After TBI,
the patient has donor marrow back through a dri p into bloodstream. Doctors
sometimes use radiotherapy to treat AML that has spread to the central nervous
system (CNS). TBI dose is given bid 3 days with a total dose of 1200cGy
(Principles and Practice of Radiation Therapy by Washington, Chp 27).
Clinical Trials: A randomized study from Seattle in the setting of AML compared
single-dose TBI (10 Gy) to a fractionated schedule (2 Gy for six fractions). The
last update of this trial showed significant superiorit y of the fractionated scheme
in terms of event -free survival. Investigators from France and from Ital y reported
that dose rate did not influence the relapse rate. Another Seatt le randomized trial
of AML in first remission compared fractionated TBI doses of 12 Gy with 15.75
Gy, showing a decreased relapse rate from 35% to 12%, but at the expense of a
significant increase in therapy-related mortality, resulting in no survival
advantage to a higher radiation dose (Encyclopedia of radiation oncology by
Brady). Thomas and colleagues were the first to compare single fraction 10Gy TBI
to 12Gy in 6 fractions treatment outcomes. Disease free survivals were
significantl y higher for fractionated TBI patients (Clinical Radiation Oncology, Pg
358)
Acute Myeloid Leukemia, Childhood
Description:AML is the second most common form of leukemia in children after
acute l ymphoblastic leukemia. AML is primaril y a cancer of the bone marrow and
lymph nodes. In AML the bone marrow malfunctions as a result of the disease
(http://www.cancer. gov/cancertopics/pdq/treatment/childAML/HealthProfessional )
Methods of Tx: chemotherapy, radiation therapy and bone marrow transplant.
RT Dose: The role of CNS prophylaxis is not well defined for AML, particularl y
as CNS relapse rates are relativel y infrequent (at about 5% to 10%). Some studies
show no difference in relapse rates with cranial radiation. Nevertheless, many
pediatric regimens employ IT drugs such as ara-C. Patients with a high WBC
count at diagnosis or monocytic variants of AML are believed to have a higher
risk for CNS relapse, which may justify both IT chemotherapy and cranial
radiation in this setting. At least one study of childhood AML demonstrated a
lower systemic relapse rate after cranial radiation.
Clinical Trials: Reduction of the prophalatic cranial radiation terapy from 24Gy to
18 Gy was tested successfull y in the Childrens Cancer Group Trial # 143. To
further reduce the potential for morbidit y f rom cranial radiation a reduction of
dose to 12 Gy or hyperfractionation like strategies are utilized (Textbook of
Radiation Oncology, by Liebel and Phillips, Chapter:54)
Chronic Myelogenous Leukemia:
Definition: CML is a chronic myeloproliferative disorder that arises from clonal
expansion of the primitive hematopoietic stem cell. It involves myeloid, erythroid,
megakaryocytic, and sometimes l ymphoid elements
(https://www.lwwoncology. com/Textbook/Content.aspx?aid=12109022).
Methods of treatment : Radiotherapy, Chemotherapy and Bone marrow transplant
RT Dose: The first effective therapy for chronic leukemias was radiotherapy to the
spleen and sometimes the liver, initiated in 1902 by Pusey and assessed in 1924 by
Minot et al.Now radiotherapy is primaril y used in a palliative setting to relieve
painful splenomegal y or other extramedullary sites when indicated. In some
centers, TBI plays an important role in allogeneic transplantation. On various case
reports in the literature, symptomatic problems from chloromas or leukemic
infiltration of the spleen may be readil y relieved with doses of 10 to 20 Gy. The
fractionation and total dose needs to take into consideration any normal tissue
toxicities and the potential for future TBI, should the patient be a potential
candidate for an allotransplant (Encyclopedia of Radiation Oncology by Brady)
Clinical Trials: A significant doserate effect has been found for CML in chronic
phase treated with allotransplant. A higher dose rate correlated with a decreased
relapse rate. As well, a multi -institutional nonrandomized French study of 180
patients with CML showed that TBI fractionation was associated with an increase
in relapse rate. Dose fractionations for clinical trails include 12Gy of TBI in 3
fractions, 10Gy in a single fraction over 4 hours and 12 Gy/fx with no lung shield
(Clinical Radiation Oncology, Pg 359)
Chronic Lymphocytic Leukemia:
Description: About 95% of cases of CLL are B cell in origin, with the remainder
having a T-cell origin. The course of the disease demonstrates marked variability.
Many patients live a normal lifespan, never require therapy, and die of unrelated
causes. The disease of others progresses within a few years despite treatment. The
usual course is characterized by gradual progression from no significant physical
findings to l ymphadenopathy, gradual increase in peripheral blood count with
increasing splenomegaly, sometimes massive in size. Nonl ymphoid organ
involvement may occur with advanced stage of disease. As disease progression
occurs, anemia and thrombocytopenia occur. Cll is nearl y twice as common as
CML. Lymphadenopathy may be present in CLL and spleen is always enlarged.
(http://emedicine.medscape.com/article/1201870-overview)
Treatment Methods: Chemotherapy and Radiotherapy, Newer approaches to the
treatment of CLL involve vaccines, cell cycle inhibitors, differentiating agent s,
and monoclonal antibodies.
Coventional RT Dose: Historicall y, low-dose TBI without stem cell support was
used to control CLL. Remarkabl y, low doses on the order of several Gray can be
effective for palliation, although fractionated doses up to 20 Gy ar e also
reasonable. Now radiotherapy is used in the management of painful splenomegal y
or occasionally for cytopenias associated with splenomegal y when splenectomy is
not an option. Doses can also vary from 4 to 10 Gy in 1 Gy/fx. Palliative radiation
therapy can also be in the form of AP/PA beams delivering 500cGy in 100cGy/fx
for 5 days. (Principles and Practice of radiation Therapy). Radiotherapy is
indicated if there is manifesting disease with adenopathy with non-l ymphoid
involvement. Chemotehrapy is administered for progressive anemia and
thrombocytopenia. The chemo drugs include chlorambucil and prednisone.
Hairy Cell Leukemia
Definition: Hairy cell leukemia (HCL) is a rare t ype of mature B-cell malignancy
of the blood and bone marrow. Body produces a surplus of abnormal B-
lymphocytes that do not function properl y. These abnormal cells can take up the
space of healthy B-l ymphocytes causing a low white blood cell count, which can
weaken the immune system and lead to infections. While the production of
abnormal B-l ymphocytes is the hallmark of this disease, the increase production of
these abnormal cells can cause a decrease in red blood cells as well as platelets.
HCL gets its name because the abnormal white blood cell s look hairy under a
microscope (Encyclopedia of Radiation Oncology)
Methods of treatment : Chemotherapy, Immunotherapy, bone marrow transplants
(rare), no radiation therapy

AIDS related lymphoma
Definition: AIDS-related l ymphoma is a disease in which malignant (cancer) cells
form in the l ymph system of patients who have acquired immunodeficiency
syndrome (AIDS). It grows and spreads very fast. AIDS related lymphomas are
intermediate or high stage and are usuall y very aggressive.
Treatment Methods: Chemotherapy, High dose Chemotherapy with stem cell
transplant, clinical trials of monoclonal antibodies. Treatment of AIDS-
related primary central nervous system l ymphoma is usuall y radiation therapy.
RT Tx: as these patients have AIDS, radiation therapy is not well defined.
Sometimes radiation therapy can be used for these patients as palliative treatment
or pain relief. If the patient has stage 3 pre-auricular nodular node involvement
than a prophylactic dose of 3600cGy can be administered with lateral fields.
Cutaneous T-cell lymphoma:
Definition: Cutaneous T-cell lymphoma (CTCL) refers to a spectrum of closel y
related malignant T-cell lymphoproliferative disorders in which the predominant
clinical manifestations involve the skin. It resembles inflammatory conditions and
remains localized to skin for long periods. It includes mycosis fungoides.
Method of Treatment : Radiotherapy(TSET)
Conventional RT dose: Superficial i rradiation (80 to 140 kVp) with a half -value
layer of 0.7 to 1 mm aluminum and a target skin distance of 15 to 30 cm can be
used for most infiltrated plaques. For markedl y infiltrated plaques and tumors,
higher-energy orthovoltage irradiation (200 to 280 kVp) or local-field electron
beam irradiation (10 to 15 MeV) is recommended. Discrete lesions may be treated
satisfactoril y with a variet y of protractionfractionation schedules, ranging from
10 to 12 Gy in three or four treatment fractions during a 3- to 4-day period, up to
20 to 30 Gy in 10 to 15 fractions during 2 to 3 weeks. The entire wide -field skin
surface receives 1.5 to 2 Gy each 2-day cycle. The majority of patients can
tolerate 2 Gy per cycle. However, patients with previous course of TSET
irradiation or atrophic skin tolerate 1. 5 Gy per cycle better. Irradiation usuall y is
administered on a 4-day/week dose schedule; the total dose depends on the intent
(curative versus palliative). Doses of 30 to 40 Gy are delivered during an 8 - to 10-
week interval with a 1- to 2-week break at 18 to 20 Gy for patients treated with
curative intent; 10 to 20 Gy is administered for palliation (Perez and Bradys
Principle and Practices of Radiation Oncology)
Clinical Trials: Several groups have reported on t he feasibility of combining
TSET irradiation with concomitant or sequential administration of cytotoxic
chemotherapy.
Hodgkins Lymphoma, Adult
Definition: Hodgkins lymphoma is rare in children younger than 10 years. The
incidence of Hodgkins increases with age and the median age of patients are
usually 26 years. Adult Hodgkins l ymphoma generall y develops in the l ymph
nodes (Perez and Bradys Principles and Practice of Radiation Therapy). The
cause of Hodgkins lymphoma is remains a mystery to researchers. It usuall y
appears as a painless mass in the neck and supraclavicular region.
Methods of Tx: Radiation therapy is the most effective single therapeutic agent for
treating Hodgkin l ymphoma. The initial successful drug combination for treating
Hodgkin l ymphoma was nitrogen mustard, vincristine, procarbazine, and
prednisone (MOPP), reported by DeVita et alfrom the National Cancer Institut e.
Combined-modalit y therapy has become the most common form of general
management for patients with Hodgkin l ymphoma. Important considerations in
combined-modalit y therapy include the sequence of therapy, the selection of
irradiation fields, the decision to irradiate all involved sites or only bulky sites,
the prescription of dose, and potential overlapping toxicities. It i s logical to
initiate treatment with chemotherapy. This has the advantages of treating all sites
of disease at the outset and reducing bulky disease to facilitate subsequent
irradiation. The irradiation dose used in combined-modalit y studies in adults
ranges from 20 to 36 Gy.
Radiation Dose: Curative doses with involved areas are 35-44Gy and uninvolved
areas are 30-36 Gy (Clinical radiation Oncology). Combination of Radiation
therapy and chemotherapy has shown to effectivel y treat earl y stage Hodgkins. If
radiation therapy is used then the patient is treated with extended field irradiation.
A dose of 3500 to 4400cGy is delivered by 6 or 15MV photons using a mantle and
paraaortic fields with a minimum dose of 3000 to 3600cGy. Typical fractionation
is 750 to 200 cGy per day depending on tolerance.
Clinical Trials: In the uncommon situations when radiation therapy alone is used
for the treatment of Hodgkin l ymphoma, the National Cancer Center Network
(NCCN) guidelines recommend a dose of 30 to 44 Gy fractionated at a rate of 7.5
to 10 Gy per week to involved sites. Because this situation is most common with
the lymphocyte predominance histology, a dose of 30 to 36 Gy is usuall y
sufficient. Evenly weighted opposed-field treatments, all fields treated dail y wit h
fractions of 1. 5 to 1. 8 Gy, depending on field size and patient tolerance, were the
general treatment parameters. A study by the German Hodgkin Study Group
(GHSG) concluded that 30 Gy was an adequate dose for prophylactic fields . In the
GHSG HD15 t ri al chemot herapy i s fol l owed by 30-Gy i rradi at i on t o PET-
posi t i ve resi dual di sease. The HD13 trial of the GHSG retains a dose of 30 Gy
for involved field treatment. The current nonrandomized Stanford G5 study
includes onl y 8 weeks of chemotherapy followed by 20 Gy involved field
irradiation (textbook of Radiation Oncology by Liebel and Phillips). Other
investigations have used dose fractionations of 30 Gy to uninvolved nodal sites
and 36 to 40 Gy in involved sites. Radiation of combined modality treatments var y
from 25Gy to 40Gy (Principles and Practice of Radiation Therapy by Washington,
Chp 26).
Hodgkins Lymphoma, childhood:
Description: Childhood Hodgkin l ymphoma is one of the few pediatric
malignancies that is similar to adult cancer. As the intial treatment was modeled
based on adults treatment, a substantial morbidity resulted. Thus, new strategies
utilizing chemotherapy and lower -dose radiation were developed. Approximately
90% to 95% of children with Hodgkin l ymphoma can be cured, prompting
increased attention to devising therapy that produces less long-term morbidit y for
these patients. Presently the treatment regimen mainl y includes chemotherapy with
low dose radiation therapy. High dose and extended fields are seldom used for
pediatric cases because of long term side effects, toxicities and secondary
malignancies
(http://www.cancer. gov/cancertopics/pdq/treatment/childhodgkins/HealthProfessio
nal/page1)
Methods of Tx: chemotherapy with radiotherapy
RT dose: Most contemporary programs for the management of pediatric Hodgkin
lymphoma are based on clinical staging and use chemotherapy alone or combined -
modality therapy, with low-dose irradiation, as higher doses of irradiation are
associated with unacceptable risks for growth impairment and late effects. To
limit growth effects, irradiation doses should not exceed 15 to 25 cGy. Low
radiation dose with chemotherapy regimens of MOPP have proved to be highl y
effective (Principles and Practice of radiation therapy) & (Textbook of Radiation
Oncology).


Hodgkins lymphoma during pregnancy
Description: Hodgkins lymphoma mainl y affects young individuals in their teens
and twenties. Many young adult women may get pregnant at this age. So, a
diagnosis of Hodgkins while a young woman is pregnant is not an unusual
condition.
Methods of Tx: As Hodgkins l ymphoma spreads slowl y, a pregnant lady is made
to wait for treatment until the conception (cancer. gov)
RT Tx: If Hodgkins is diagnosed during the first half of pregnancy, the intention
is to delay the treatment if possible to prevent any damage to the fetus.
Chemotherapy that is administered in the first trimester has been associated with
congenital abnormalities in as many as 33% of infants. Radiation is not
recommended during pregnancy.

Non-Hodginks Lymphoma, childhood
Description: Childhood non-Hodgkins l ymphomas are a heterogeneous group of
malignancies with variable histopathology, site of origin, and clinical
manifestations. Childhood NHLs are diffuse, high-grade, and poorly
differentiated; extranodal involvement is common and dissemination occurs earl y
and often. This is in striking contrast to the adult NHL where low- and
intermediate-grade nodal disease predominates. NHL may arise anywhere ethat the
lymph fluid can travel. It can develop in l ymph node and a region of l ymph node,
in an organ or a combination of both. NHL is more common in older people at the
age or above 65 years.
Methods of Tx: Chemotherapy, Radiotherapy, new biologic therapies and stem cell
transplants
RT Tx and Dose: It is reserved for emergency treatment of mediastinal disease or
spinal cord compression, treatment for patients who fail to obtain a complete
remission after induction chemotherapy, palliation of pain or mass effect,
consolidation before bone marrow transplantation Local residual disease, after
induction chemotherapy or as relapse after a complete remission, often most is
managed with local field irradiation in doses ranging from 30 Gy for the small cell
lymphocyte/l ymphoblastic to 45 Gy for the large cell histiocytic subtypes.
Palliation of cranial nerve deficits, however, requires higher total doses of local
field irradiation (20 to 30 Gy). To reduce the potential adverse effects assoc iated
with radiation therapy, protocols reduced the local field dose to 20 Gy and the
volume of tumor margin from 5 cm to 2 to 3 cm (Textbook of Radiation
Oncology).

NonHodgkins Lymphoma, Adult
Description: Non-Hodgkins l ymphoma (NHL) is a heterogeneous group of
malignancies of the lymphoid system characterized by an abnormal clonal
proliferation of B cells, T cells, or both (cancer. gov)
Methods of Tx: Surgery, Chemotherapy, radiation Therapy and Immunotherapy
RT Dose/fx: There was a suggestion that tumor bulk appeared to influence the
outcome. Patients with tumor size >6 cm were treated with combined modalit y
therapy (CMT). It was suggested that doses of at least 40 Gy were necessary in
these circumstances for optimal local control, although the data demonstrate local
failure in 1 of 51 patients treated with more than 40 Gy as part of a CMT program
versus 4 of 70 patients treated with doses of 30 to 40 Gy. It is unclear if tumor
size affects the dose required for local control. Smaller tumors may do well with
<30 Gy. Doses of 35 to 45 Gy are recommended for local control. Intermediate
grade l ymphomas can be curativel y treated with chemo followed by 30 to 35 Gy of
radiotherapy (Textbook of Radiation Oncology, Chp 61).
Clinical Trials: Investigators at the M.D. Anderson Cancer Center examined local
control in 294 patients with stages I to IV DLBCL after CHOP-based
chemotherapy in a retrospective review. Because of varying dose fractionation
schemes, radiation doses were converted using the linear quadratic model to
biologicall y equivalent doses given at 1.8 Gy per fraction. Patients were then
grouped in the 30- to 40-Gy range and 40- to 50-Gy range. The authors suggested
that doses >40 Gy might be needed for large tumors.
Primary CNS lymphoma
Description: Primary CNS lymphoma (PCNSL) is an aggressive malignancy
arising exclusivel y in the CNS, that is, the brain parenchyma, spinal cord, eyes,
cranial nerves, and/or meninges. 1 This lymphoma represents 4% of intracranial
neoplasms and 4%-6% of all extranodal l ymphomas
Methods of Tx: Chemotherapy and radiotherapy
RT Tx and Dose: PCNSL is a radiosensitive tumor and radiotherapy has been the
standard treatment for decades. In these patients, the whole brain should be
irradiated because of the diffuse infiltrative nature of PCNSL and the inclusion of
the eyes within the radiation volume is suggested. The optimal dose of WBRT is
controversial, but a dose of 40-50 Gy has been suggested. Doses > 50 Gy and the
addition of a boost are associated with increased risk of neurotoxicity. WBRT 40-
50 Gy appears advisable as exclusive treatment when chemotherapy is
contraindicated (http://bloodjournal.hematologylibrary. org/content/118/3/510.full )

Sezary Syndrome
Description: Sezary Syndrome is a t ype of cutaneous T-cell l ymphoma. The
affected T-cells have pathological amount of mucopoll ysaccarides. It is considered
the late stage of mycosis fungoides with lymphadenopathy.
Methods of Tx: Chemotherapy, Radiation therapy and phototherapy
RT dose/fx: It is generall y acknowledged that TSEBT should be used in
conjunction with multimodality therapy if nodal, blood, or visceral involvement is
present unless palliation alone is the objective. The best results with TSEBT are
based on a highl y fractionated regimen of 32 to 36 Gy with appropriate shielding
as per standard protocol. A fractionated regimen over 9 weeks is recommended in
an effort to minimize both acute and chronic effects.
Clinical Trials: 20-30Gy (Jones GW, Rosenthal D, Wilson LD) Total skin electron
radiation for patients with erythrodermic cutaneous T-cell l ymphoma (mycosis
fungoides and the Sezary syndrome) Cancer 1999;85:1985-95. ), Combined
chemotherapy with TBERT of 28-32Gy (Duvic M, Apisarnthanarax N, Cohen DS,
Smith TL, Ha CS, Kurzrock R. Anal ysis of long-term outcomes of combined
modality therapy for cutaneous T-cell l ymphoma. J Am Acad Dermatol
2003;49:35-49.)
Waldenstrm's macroglobulinemia
Description: Macroglobulinemia of Waldenstrom is a cancer of the B
lymphocytes. It is associated with the overproduction of proteins called IgM
antibodies.
Methods of Tx: Plasmapheresis removes unwanted substances from the blood. In
macroglobulinemia, it removes or reduces the high level of IgM, and is used to
quickl y control the symptoms caused by blood thickening. Drug therapy may
include steroids or combinations of chemotherapy drugs. Patients who have a low
number of red or white blood cells or platelets may need transfusions or
antibiotics.
Chronic Myeloproleferative disorder
Description: Chronic myeloproliferative disorders are a group of slow-growing
blood cancers in which too many abnormal red blood cells, WBCs, or platelets,
which circulate in the blood. The t ype of myeloproliferative disorder is based on
whether too many red blood cells, white blood cells, or platelets are being made.
Body can start making too many of the blood types , but usuall y one t ype of blood
cell is affected more than the others are
(http://rarediseases.info.nih. gov/gard/9319/chronic-myeloproliferative-
disorders/resources/1)
Methods of Tx: Transfusion therapy, Chemotherapy, Radiation therapy, Surgery,
Immunotherapy, chemotherapy with stem cell transplant.
Langerhans Cell Histiocytosis
Description: Langerhans cell histiocytosis (LCH) is the most common of the
histiocytic disorders and occurs when the body accumulates too many immature
Langerhans cells. Langerhans cells are a type of white blood cell that normall y
helps the body fight infection. In LCH, too many Langerhans cells are produced
and build up in certain parts of the body where they can form tumors or damage
organs (http://www.histio.org/lchinchildren)
Methods of Tx: Medications, Chemotherapy, radiation therapy
RT Dose: 15Gy in 2Gy/fx, low dose shows good local control, in the treatment of
adults doses from 10 to 20 Gy are recommended

Plasma Cell Neoplasm:
Description: Plasma cell neoplasms are diseases in which the body makes too
much plasma cells. Plasma cell neoplasms account for 22% of all mature B-cell
neoplasms. The most common t ype of plasma cell neoplasm is multiple myeloma.
Methods of Tx: Chemotherapy and Radiation therapy
RT Dose: A phase III French study (trial 9502) examined melphalan alone versus
melphalan with TBI, 8 Gy in four fractions and found that patients in the TBI -
containing arm suffered more grade 3 or 4 mucosal toxicity. Diffuse bone pain
involving wide areas of the skeleton can be effectivel y palliated by half body
radiation with single doses of 5 to 8 Gy. When RT is given for pain due to disease
involving a long bone, a local field suffices. Doses of 10 to 20 Gy (in five to 10
fractions) are effective, although the pain relief i s often partial (Clinical Radiation
Oncology).
Clincal Trials: A multicenter study suggested that a longer fractionated regimen
(30 Gy in 10 fractions or higher) was associated with better neurologic recovery
than 20 Gy in five fractions or a single 8 Gy f raction.
Myelodysplastic Syndrome
Description: Myelodysplastic syndromes (MDS) are conditions that occur when
the blood-forming cells in the bone marrow are damaged. The damage of blood
forming cells leads to deficiency of one or more t ypes of blood and blood count.
In MDS, some of the new blood cell forming bone marrows are damaged and do
not function properl y. Many of the blood cells formed by the damaged bone
marrow cells are defective. Defective cells often die earlier than normal cells . Our
body destroys the abnormal blood cells, leaving the patient with low blood counts
because there arent enough normal blood cells
(http://www.cancer.org/cancer/myelodysplasticsyndrome/overviewguide/myelodys
plastic-syndromes-overview-what-is-myelodysplastic-syndrome)

Methods of Tx: Blood transfusion, Chemotherapy and Bone marrow transplant

RT Tx and Dose: Stem cell transplant is the onl y treatment that can cure
myelodysplastic syndrome (MDS). In this treatment, the patient receives high -dose
chemotherapy and/or total body irradiation to kill the cells in the bone marrow
(including the abnormal bone marrow cells). Then the patient receives new,
healthy blood-forming stem cells.