Clinical Manifestations in Hematology

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Questions and Answers

Which of the following is a clinical manifestation associated with severe neutropenia?

Increased appetite

Persistent headaches

Easy bruising (correct)

Frequent urination

What characteristic is commonly associated with Hodgkin's disease?

Higher prevalence in patients over 70

Common in patients with HIV

Presence of Reed-Sternberg cells (correct)

Formation of multiple lymphomatous lesions

Which type of Non-Hodgkin's lymphoma is known for its association with the overproduction of immature leukocytes?

Acute Lymphocytic Leukemia (correct)

Follicular Lymphoma

Chronic Lymphocytic Leukemia

Hairy Cell Leukemia

What is typically used as a supportive care strategy in patients diagnosed with Disseminated Intravascular Coagulation (DIC) who are not actively bleeding?

Monitoring and treating the underlying disease Signup and view all the answers

Which of the following is NOT a part of the diagnostic tests for DIC?

Serum creatinine level Signup and view all the answers

What characterizes acute DIC?

It leads to severe consumptive coagulopathy. Signup and view all the answers

Which manifestation is NOT associated with bleeding from DIC?

Pulmonary emboli Signup and view all the answers

Which symptom suggests thrombotic changes due to DIC?

Tachypnea Signup and view all the answers

What underlying condition is crucial for DIC resolution?

Resolving the underlying disease. Signup and view all the answers

Which of the following is NOT a neurological manifestation of DIC?

Bone and joint pain Signup and view all the answers

What type of DIC is frequently observed in patients with solid tumors?

Chronic DIC Signup and view all the answers

What clinical manifestation indicates a severe state of DIC affecting the integumentary system?

Hematomas Signup and view all the answers

Which of the following is a thrombotic manifestation of DIC?

Ischemic tissue necrosis Signup and view all the answers

Which clinical manifestation is most commonly associated with multiple myeloma?

Bone pain in ribs and spine Signup and view all the answers

What is a potential complication of splenectomy?

Increased infection risk Signup and view all the answers

In hemophilia A, which factor is typically deficient?

Factor VIII Signup and view all the answers

What is a primary treatment method for hereditary hemochromatosis?

Phlebotomy Signup and view all the answers

What is a key clinical feature of von Willebrand’s disease?

Prolonged bleeding times Signup and view all the answers

What is a primary treatment for bleeding episodes in patients with hemophilia?

Cryoprecipitate prior to surgery Signup and view all the answers

What is a known potential risk factor for developing acquired hemochromatosis?

Alcohol abuse Signup and view all the answers

Study Notes

Disseminated Intravascular Coagulation (DIC)

DIC is a serious disorder involving bleeding and blood clots.
It results from abnormally fast clotting and anticlotting processes in response to disease or injury.
DIC is characterized by excessive bleeding due to depleted platelets and clotting factors.
An underlying disease or condition always causes DIC.
Treatment focuses on treating the underlying cause to resolve DIC.

Pathogenesis of DIC

Widespread activation of coagulation
Endothelial damage
Generalized platelet aggregation
Microthrombi form in the circulation, blocking blood flow.

Events Leading to Thrombosis and Bleeding in DIC

Circulating thrombi obstruct microcirculation in organs.
Fibrinolysis in microcirculation
Consumption of platelets, AT III, fibrinogen, and clotting factors
Consumption of coagulation proteins

Bleeding Manifestations

Integumentary: Pallor, petechiae, purpura, oozing blood, hematomas
Respiratory: Hemoptysis
Cardiovascular: Hypotension
Gastrointestinal: Upper and lower GI bleeding, abdominal distension
Bloody stools, hematuria, changes in mental status, musculoskeletal complaints (bone and joint pain).

Thrombotic Manifestations

Fibrin or platelet deposition in microvasculature
Integumentary changes (e.g., cyanosis)
Ischemic tissue necrosis (e.g., gangrene, hemorrhagic necrosis)
Respiratory changes (e.g., tachypnea, dyspnea)
Pulmonary emboli
Acute respiratory distress syndrome (ARDS)
Cardiovascular changes (ECG changes, venous distension)
Gastrointestinal changes (abdominal pain, paralytic ileus)
Urinary changes (oliguria leading to renal failure)

Acute vs Chronic DIC

Acute DIC: Develops suddenly, severe consumptive coagulopathy leading to hemorrhage, and frequent organ failure.
Chronic DIC: Reflects a compensated state, more frequently observed in solid tumors and large aortic aneurysms, and clinical manifestations vary from easy bruising to thrombosis.

Diagnostic Tests for DIC

Prolonged PT, PTT, APTT
Reduced platelets, fibrinogen
Fibrin split products (FSPs)
Factor assays (factors V, VIII, X, XIII)
D-dimers (cross-linked fibrin fragments)
Antithrombin III (AT III)
Protein C and S (antithrombotic proteins)
Plasminogen, tissue activator
Peripheral blood smear

Management of DIC

Stabilize the patient (e.g., oxygenation, volume replacement)
Treat the underlying cause of DIC.
Provide supportive care for the manifestations.
No therapy for DIC is needed if it is chronic and the patient isn't bleeding.
Treatment of the underlying disease can reverse the DIC (e.g., chemotherapy for malignancy).
When the patient bleeds, direct therapy towards providing support with blood products and treat the primary cause.

Therapy for DIC

Treat the cause.
Use Heparin and AT III.
Fresh-frozen plasma, cryoprecipitate, and red blood cell transfusions.
Platelet transfusions
Hemodialysis for acute renal failure

Leukemia

Malignant disorders affecting the blood and blood-forming tissues.
Commonly affects the bone marrow, lymph system and spleen.
Often thought of as a childhood disease
Adults are affected 10 times more than children
Characterized by diffuse replacement of bone marrow with proliferating leukocyte precursors resulting in dysfunctional cells
Progressive disease if untreated.

Clinical Manifestations of Leukemia

Symptoms result from complications of leukemic cells infiltrating organs including bone marrow.
Fatigue and weakness
Headache, mouth sores, anemia, bleeding, fever, infection
Hepatomegaly (enlarged liver)
Lymphadenopathy (enlarged lymph nodes)
Bone pain, meningeal irritation, oral lesions (chloromas)
Marrow-suppression

Diagnostics for Leukemia

Low RBC count, Hb, and Hct; low platelet count; low to high WBC count (myeloblasts); high LDH; greatly hypercellular bone marrow with myeloblasts
Peripheral blood evaluation
Bone marrow examination (morphological, histochemical, immunological, and cytogenetic methods)
Lumbar puncture and CT scan to see presence of leukemic cells outside the blood and bone marrow
Specific cytogenetic abnormalities

Care Management for Leukemia

Addressing physical and psychosocial needs of the patient, and their family.
Addressing comorbid conditions affecting treatment decisions
Maximizing patient's physical functioning
Teaching patients that adverse effects of treatment are usually temporary
Encouraging patients to discuss quality-of-life issues
Assessing laboratory data
Being knowledgeable about all medications

Chemotherapy for Leukemia

Combination chemotherapy used as the mainstream treatment.
Has 3 purposes: overcoming drug resistance
reducing drug toxicity by using multiple drugs for varying toxicities
interrupting cell growth points during the cell cycle
Specific chemotherapy regimens, CBC check-ups, and schedules vary according to each patient and their cycle.

Bone Marrow & Stem Cell Transplantation for Leukemia

Aims to totally eliminate leukemic cells from the body.
Uses combinations of chemotherapy, sometimes with whole body irradiation, to achieve elimination.
Replaced with healthy cells from an HLA matched donor.
Possible options include siblings, volunteers, identical twins, or patient's own stem cells removed beforehand via autologous or allogeneic procedures.

Chemotherapy for Leukemia - Categories and Examples

Alkylating agents: Damage DNA (Cisplatin, Adriamycin)
Antitumor Antibiotics: DNA synthesis inhibition (Methotrexate, Docetaxel)
Antimetabolites: Impeding cell cycle (Vincristine)
Taxanes: Interrupt cell cycle (Imatinib)
Monoclonal Antibodies: Antibody targeting specific proteins (Rituximab, Trastuzumab)
Tyrosine Kinase Inhibitors: Inhibit tyrosine enzymes (Imatinib)

Adverse Reactions to Leukemia Treatment

Antineoplastic medication causes rapid destruction of normal cells, resulting in increased uric acid.
Mucositis, alopecia, anorexia, nausea and vomiting, diarrhea, anemia, neutropenia, thrombocytopenia, and neuropathy.

Neutropenia

Decreased neutrophils
Increased risk of infection
Can be gradual or sudden
Resulting from other conditions or diseases
Drug therapy, hematological disorders, autoimmune disorders, infections, or hemodialysis
Clinical manifestations include missing signs of infection and sudden presentation of septic shock

Clinical Manifestations of Neutropenia

Signs of inflammation (redness, heat, swelling) may not be present.
Absence of pus formation.
Low-grade fever can indicate infection and lead to septic shock.
Fever > 38°C and neutrophil count < 0.5 x 10⁹/L is a medical emergency.

Hodgkin's Lymphoma

Malignant lymphoma characterized by proliferation of abnormal giant, multinucleated Reed-Sternberg cells.
Located in lymph nodes.

Clinical Manifestations of Hodgkin's Lymphoma

Insidious onset, with lymph node enlargement (movable, nontender)
B-symptoms (fever, night sweats, weight loss).
Advanced stages include hepatomegaly, splenomegaly, or anemia depending on location.
Depending on the site, symptoms may include superior vena cava syndrome or abdominal masses interfering with renal function if retroperitoneal and/or jaundice from liver involvement.
Bone involvement might lead to bone pain
Spinal cord compression potentially leading to paraplegia.

Staging of Hodgkin's Lymphoma

Stage I: Involvement of one lymph node group on one side of the diaphragm.
Stage II: Involvement of two or more lymph node groups on one side of diaphragm.
Stage III: Involvement of lymph node groups on both sides of the diaphragm.
Stage IV: Spread to organs or bone marrow.

Treatment of Hodgkin's Lymphoma

Radiation therapy is effective in the majority of patients in stages I and II.
Combination chemotherapy (e.g., MOPP and ABVD) is used for advanced stages.

Non-Hodgkin's Lymphoma (NHL)

Varies from slowly developing to rapidly progressive lymphomas.
Types include Burkitt's, diffuse large B-cell, and follicular lymphoma.
Accounts for 90% of lymphomas are B-cells and 10% of lymphomas are T-cells.
No single hallmark.
NHL involves lymphocytes arrested at various stages of development.

Collaborative Care for NHL

Treatment is usually chemotherapy +/- radiation.
More aggressive lymphomas are more responsive to treatment.
Chemotherapy regimens include CHOP, CVP, and COPP.
Biological therapy options include alpha interferon, rituximab, and ibritumomab tiuxetan (Zevalin).

Complete Remission in NHL

Complete remissions are uncommon
Most respond with improvement and alleviation of adenopathy and symptoms.
Radiation alone can be sufficient for localized stage I or II disease.
For advanced disease, "watchful waiting" approach to assess disease progress.

Multiple Myeloma

Malignant tumor of plasma cells within the bone marrow, arising from a single clone.
Accounts for more than 40% of malignant tumors of the bone.

Clinical Manifestations of Multiple Myeloma

Bone pain, especially ribs, spine, and pelvis
Weakness and fatigue
Recurrent infections
Urinalysis shows Bence Jones proteinuria
Osteoporosis
Elevated calcium and uric acid levels
Kidney failure
Spinal cord compression

Disorders of the Spleen

Spleen enlargement can cause sequestration of blood products.
Removal of the spleen predisposes to infection potential.
Rupture may cause radiating pain

Hemochromatosis

Hereditary genetic condition where excessive iron is deposited in organs.
No evident signs of disease until middle age.
Men have more complications than women.
Treatment is phlebotomy
Can have acquired form, common in alcoholics

Hemophilia

Two inherited disorders (A and B).
Hemophilia A caused by lack of factor VIII.
Hemophilia B (Christmas disease) caused by lack of factor IX.
Men get this disease while women are carriers.
The main issue is bleeding, especially into joints, resulting in swelling and pain.
Can bleed with or without trauma.

von Willebrand Disease

Common bleeding disorder that affects males and females equally.
von Willebrand factor (vWF) is needed for the proper activity of factor VIII.
Can lead to bleeding, especially nosebleeds, excessive bleeding from cuts, heavy menstruation, and postoperative bleeding issues.
Prolonged bleeding times are common.
Treatment includes treatment (similar to hemophilia) and DDAVP (increases factor VIII).
Cryoprecipitate may be required prior to major surgeries.

Thrombocytopenia

Reduction of platelets below 150 x 10⁹/L
Immune thrombocytopenic purpura
Heparin-induced thrombocytopenia.

Apheresis

AKA Plasmapheresis; removes blood components through centrifuge
Can obtain platelets, white blood cells, or harvest stem cells for transplant.

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Description

Test your knowledge on clinical manifestations associated with hematological disorders, including neutropenia, Hodgkin's disease, and Non-Hodgkin's lymphoma. This quiz will assess your understanding of diagnostic tests and supportive care strategies in these conditions.