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Study Guides:

I Wish I Knew Earlier

Here are some points that can make your Hematology/Oncology life easier- The earlier you learn them, the better. Lesson Number 1: Below is a list of anticancer agents for which the category and mechanism of action can be recognized by their names:

White Cell Disorders

Myeloid cells: Granulocytes (neutrophils, eosinophils, basophils) Neutrophils maturation in BM: 7-10 days, circulates in blood: 1 day, duration in tissue: 2-3 days Monocytes Macrophages Gaucher’s Disease:  Autosomal recessive lysosomal storage disorder Caused by a deficiency of the enzyme glucocerebrosidase (GBA gene) Cells accumulate in the liver, spleen, and bone marrow Classic “crumpled tissue paper” appearance (Buzz word) Dendritic cells Mast cells Lymphocytes: B cells T

Thyroid Carcinoma

Types: Papillary Thyroid Carcinoma Medullary Thyroid Carcinoma Follicular Thyroid Carcinoma Hurthle Cell Carcinoma Anaplastic Thyroid Carcinoma Suspicious features of thyroid nodules on US: Irregular margins Microcalcifications Taller than wide shape Rim calcifications with small extrusive soft tissue component Extrathyroidal extension Central vascularity Treatment: Treatment typically entails surgery (lobectomy vs. thyroidectomy) Indications for thyroidectomy : History of pr

Testicular Cancer

Risk factors: History of cryptorchidism (undescended testis) Family history of testicular cancer Personal history of testicular cancer Initial work up: Testicular ultrasound Tumor markers: AFP, hCG, LDH Do not biopsy testis. Radical inguinal orchiectomy (through inguinal incision, not scrotum) Brain MRI recommended if: Neurologic symptoms Post-orchiectomy beta-hCG > 5000 Extensive lung metastases Non-pulmonary visceral metastases AFP >10000 (non-seminoma) Predominant chorioca

T-Cell Non-Hodgkin Lymphoma (NHL)

Introduction: NHL is categorized into two groups of B-cell lymphomas and T-Cell lymphomas: B-cell lymphomas (85–90%): (discussed in a separate post B-Cell Non-Hodgkin lymphoma ) Low-grade B-cell lymphomas (LGBL): DLBCL: The most common, aggressive Follicular lymphoma (FL): Indolent, arising from germinal center B cells Marginal zone lymphoma (MZL): Extranodal, Nodal, Splenic Mantel cell lymphoma (MCL): More aggressive, Derived from mantle zone B cells Chronic lymphocytic leu

Soft Tissue Sarcoma

Background: Genetic Risks: Li-Fraumeni syndrome (p53) NF1 (malignant peripheral nerve sheath tumors) Familial Rb Gardeners/FAP (APC) Desmoid tumors Most are sporadic tumors Risk factors: Prior radiation is the #1 risk factor (esp within 10-30 years) HIV or HHV-8 (Kaposi Sarcoma) Chronic Lymphedema (Angiosarcoma) Most common types: Gastrointestinal stromal tumor (GIST) Characterized by c-KIT or PDGFRA mutations Liposarcoma Well-differentiated/Dedifferentiated liposarcoma MDM2

Small Cell Lung Cancer (SCLC)

Background:  Poor prognosis. Median OS ~ 12-14 month Limited-Stage SCLC: confined to the ipsilateral hemithorax, which can be safely encompassed within a radiation field Extensive-Stage SCLC: metastatic, beyond the ipsilateral hemithorax, including malignant pleural or pericardial effusion May present with SVC Syndrome IR consult - Ideally could still get tissue diagnosis before treating Rad Onc consult for Emergent treatment with concurrent chemoRT LS-SCLC Treatment Stage I-

Skin Cancer

Cutaneous Melanoma The BRAF V600 mutation is a frequent somatic/acquired driver mutation in cutaneous melanoma. Cyclin-dependent kinase inhibitor 2A (CDKN2A) is the most frequently mutated high-risk gene associated with familial cutaneous melanoma. Staging: Any N+ disease is Stage IIIA and up Memorize staging: Stage Thickness Ulceration T1a < 0.8 mm No T1b < 0.8 mm Yes T1b 0.8–1 mm with or without T2a 1–2 mm No T2b 1–2 mm Yes T3a 2–4 mm No T3b 2–4 mm Yes T4a > 4 mm No T4b > 4

Red Cell Disorders

Hemoglobinopathy Sickle Cell Disease (discussed below) Thalassemia (discussed below) Hemoglobin C Substitution of lysine for glutamic acid in 6th position of the beta globin chain Subtypes: Hb AC (Hb C trait): No symptoms Hb CC (Hb C disease): Causes HbC crystals Symptoms: anemia, mild hemolytic anemia (prone to gallstone), splenomegaly Hb SC: less sickling and vaso-occlusive events compared to Hb SS More retinopathy, priapism and ischemic necrosis of bones Hemoglobin E Su

Prostate Cancer

Early detection: Individualized informed decision-making for prostate cancer screening (DRE and PSA) Discuss risks and benefits: Age 45–75 at average risk Age 40–75 at high risk (Black/African American, family history, germline mutations) Definitive diagnosis: Requires prostate biopsy, Gleason grading, and TNM staging. Clinical Pearls: Think of Prostate Cancer treatment on a "spectrum": Localized Disease → Biochemical recurrence (BCR) → Non-metastatic castrate resistant prost

Plasma Cell Disorders

Monoclonal Gammopathies IgG >> IgM > IgA SLiM-CRAB: S: ≥ 60% BM Plasmacytosis Li: K/L ratio ≥ 100 or ≤0.01 M: MRI >1 focal lesion (>5 mm size) C: Calcium >11 or >1 ULN R: Renal Cr >2 or CrCl <40  A: Anemia Hb <10 or 2< LLN B: ≥ 1 lytic lesions MGUS:  M protein <3                                           Plasma cell in BM <10%          No SLiM-CRAB SMM:    M protein ≥ 3 or ≥ 500 mg/24hr urine     Plasma cell in BM 10-60%       No SLiM-CRAB MM:      Plasma cell proliferative d

Platelet Disorders

Qualitative: Defects in membrane glycoproteins Bernard-Soulier Syndrome (GPIb/IX/V) Glanzmann Thrombasthenia (GPIIB/IIIa) Collagen Defects (GPVI and GPIa/IIa) Defect in platelet granules Storage Pool Deficiency Gray Platelet Syndrome Quebec Platelet Disorder Hermanksy Pudlak syndrome Defects in platelet secretion and signaling Defects in platelet coagulant activity Defects of transcription factors RUNX1 Defects of cytoskeletal/structural proteins Wiskott-Aldrich Syndrome B1-T

Pancreatic Cancer

Work up: EUS/ERCP for biopsy and stent placement Consult GI for ERCP if bilirubin high (prior to giving chemotherapy) CT Chest/Abdomen/Pelvis CA 19-9 (baseline) CA19-9 is a good marker but should not be used to determine need for treatment alone Elevated CA 19-9 prompts further evaluation/imaging Consider screening in patients with strong family history of pancreatic cancer and genes associated with pancreatic adenocarcinoma: ATM, BRCA1, BRCA2, CDKN2A, MLH1, MSH2, MSH6, EPCAM

Non-Small Cell Lung Cancer (NSCLC)

Background: NSCLC (85%): Adenocarcinoma (60%), Squamous (30-35%), other Neuroendocrine (5%) SCLC (15%) Staging: Recommend to memorize TNM staging (questions will not always state what stage but may provide size, nodal involvement, etc) T2 starts at 3 cm and each T goes up by 2 cm → T2 (3 cm), T3 (5cm), T4 (7cm) N1 is hilar nodes, N2 is ipsilateral mediastinal nodes, N3 is contralateral OR supraclavicular nodes Stage I NSCLC Curative intent: Surgical resection (preferred) or r

Novel Therapies For Hematologic Malignancies

Diffuse large B-cell lymphoma (DLBCL) Anti-CD19 CAR-T cell: Preferred for relapsed/refractory DLBCL if relapsed <1 year. Tisagenlecleucel (tisa-cel) Axicabtagene ciloleucel (axi-cel) Lisocabtagene maraleucel (liso-cel) Brexucabtagene autoleucel (brexu-cel) Anti-CD19 mAb: Tafasitamab Anti-CD20 mAb: Rituximab Obinutuzumab Anti-CD79b ADC ( Antibody-Drug Conjugate) : Polatuzumab vedotin Used in Pola-R-CHP regimen Anti-CD19 ADC: Loncastuximab Tesirine CD20 x CD3 Bispecific Ab: Eng

Myelodysplastic Syndrome (MDS)

Background: Diagnosis is based on morphological evidence of dysplasia in BMBx Additional studies: karyotyping, flow cytometry, molecular genetics Pearl: Regardless of blast percentage, if a characteristic AML cytogenetic abnormality is seen on BMBx, then should treat as AML. Can be associated with previous drug exposures Example: alkylating chemotherapy or topoisomerase inhibitors Associated with del 5, del 7, or complex chromosomal abnormalities Risk Stratification: Based on

Mesothelioma

Background: Types: Pleural mesothelioma (~85%) Peritoneal mesothelioma (~15%) Histology: Epithelioid Mesothelioma: Epithelioid-to-round cells Better prognosis Non-Epithelioid Mesothelioma: Sarcomatoid: Spindle cells with tapered nuclei Biphasic: Contains both epithelioid and sarcomatioid components in various proportions (each at least 10% of the tumor) Diagnosis: Immunohistochemical panels are essential for diagnosis, requires both criteria: 2 positive mesothelial markers:

Mechanism of Action

Here is a list of common anticancer therapies (chemotherapy, immunotherapy and targeted therapy), categorized by mechanism of action, along with information on their routes of administration.

Lung Cancer (Snap Review)

Non-Small Cell Lung Cancer (NSCLC) T1  T2: 3 cm T3: 5 cm T4: 7 cm N1: Hilar nodes N2: Ipsilateral N3: Contralateral or supraclavicular Stage I:  N0 Stage Ia: T1  (<3 cm, No NGS) Stage Ib: T2a (3-4 cm):              Alectinib if ALK mutated              Osimertinib if EGFR exon 19 deletion or L858R Stage II:  >N1 If unresectable:              Radiation If resectable: Alectinib if ALK mutated Osimertinib if EGFR exon 19 deletion or L858R Chemotherapy: Platinum/Taxol x4 Platinum

Lymphoma (Snap Review)

Hodgkin lymphoma (HL): B-cell lymphoma Non-Hodgkin lymphomas (NHL): B-cell lymphomas (85–90%): DLBCL: The most common, aggressive Follicular lymphoma (FL): Indolent, arising from germinal center B cells Marginal zone lymphoma (MZL): Extranodal, Nodal, Splenic Mantel cell lymphoma (MCL): More aggressive, Derived from mantle zone B cells Burkit lymphoma (BL): Highly aggressive, germinal center origin Chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) Lymphoplasm

Kidney Cancer

Genetics: Consider VHL, Tuberous sclerosis, Birt-Hogg-Dube (spontaneous pneumothorax is a hallmark) Genetic testing in patients with: Multiple renal masses, bilateral or multifocal tumors, diagnosis age < 45, family history of RCC (more than one first/second degree relatives with RCC) Types: Clear cell RCC: 75% Non-Clear Cell RCC: 25% Papillary RCC is associated with MET mutation.  Collecting Duct RCC Chromophobe RCC Associated with Birt-Hogg-Dube Medullary RCC Associated wit

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