Podcast
Questions and Answers
Which of the following is NOT a manifestation of anemia?
Which of the following is NOT a manifestation of anemia?
- Nocturnal diarrhoea (correct)
- Gustatory sweating
- Postural hypotension
- Progressive fatigue
Gustatory sweating is a common symptom associated with hematology disorders.
Gustatory sweating is a common symptom associated with hematology disorders.
True (A)
What is the main treatment mentioned for managing hematological disorders?
What is the main treatment mentioned for managing hematological disorders?
High dose IV melphalan + Dexamethasone
In males, __________ is associated with carpal tunnel syndrome.
In males, __________ is associated with carpal tunnel syndrome.
Match the following hematological manifestations with their respective descriptions:
Match the following hematological manifestations with their respective descriptions:
Which mutation is commonly associated with Waldenstrom's Macroglobulinemia?
Which mutation is commonly associated with Waldenstrom's Macroglobulinemia?
Waldenstrom's Macroglobulinemia primarily secretes IgA.
Waldenstrom's Macroglobulinemia primarily secretes IgA.
What is one clinical feature associated with hyperviscosity in Waldenstrom's Macroglobulinemia?
What is one clinical feature associated with hyperviscosity in Waldenstrom's Macroglobulinemia?
The presence of _______ is a key diagnostic criterion for Waldenstrom's Macroglobulinemia.
The presence of _______ is a key diagnostic criterion for Waldenstrom's Macroglobulinemia.
Match the following clinical features to their description:
Match the following clinical features to their description:
Which of the following is a poor prognostic factor in plasma cell disorders?
Which of the following is a poor prognostic factor in plasma cell disorders?
Daratumumab is used only for patients who are eligible for transplant.
Daratumumab is used only for patients who are eligible for transplant.
What is the induction treatment for standard-risk plasma cell disorders?
What is the induction treatment for standard-risk plasma cell disorders?
Carpal tunnel syndrome in males is a result of __________.
Carpal tunnel syndrome in males is a result of __________.
Match the following treatments with their risk categories:
Match the following treatments with their risk categories:
What characteristic distinguishes monoclonal gammopathy from polyclonal gammopathy in a serum protein electrophoresis graph?
What characteristic distinguishes monoclonal gammopathy from polyclonal gammopathy in a serum protein electrophoresis graph?
Increased levels of β2 microglobulin are a common serum marker observed in plasma cell disorders.
Increased levels of β2 microglobulin are a common serum marker observed in plasma cell disorders.
What is a common finding in the microscopic view of bone marrow in patients with plasma cell disorders?
What is a common finding in the microscopic view of bone marrow in patients with plasma cell disorders?
The deletion of chromosome 17p is classified as a ______ risk factor in Multiple Myeloma.
The deletion of chromosome 17p is classified as a ______ risk factor in Multiple Myeloma.
Match the classification of Multiple Myeloma with their risk factors:
Match the classification of Multiple Myeloma with their risk factors:
What is the most common hematological malignancy?
What is the most common hematological malignancy?
Acute kidney injury in the context of multiple myeloma can be caused by light chain cast nephropathy.
Acute kidney injury in the context of multiple myeloma can be caused by light chain cast nephropathy.
What is a common symptom associated with multiple myeloma related to bone issues?
What is a common symptom associated with multiple myeloma related to bone issues?
The primary cytogenetic abnormalities leading to multiple myeloma include mutations in Ras and abnormalities in ______.
The primary cytogenetic abnormalities leading to multiple myeloma include mutations in Ras and abnormalities in ______.
Match the following conditions with their renal effects:
Match the following conditions with their renal effects:
What is the lifespan of platelets?
What is the lifespan of platelets?
Thrombocytopenia is defined as a platelet count below 100,000/μL.
Thrombocytopenia is defined as a platelet count below 100,000/μL.
What is the increase in platelet count expected from 1 unit of single donor platelets (SDP)?
What is the increase in platelet count expected from 1 unit of single donor platelets (SDP)?
A platelet count below __________ can result in significant bleeding.
A platelet count below __________ can result in significant bleeding.
Match each type of platelet transfusion with its characteristic:
Match each type of platelet transfusion with its characteristic:
Which of the following is a major criterion for the diagnosis of POEMS syndrome?
Which of the following is a major criterion for the diagnosis of POEMS syndrome?
Vascular Endothelial Growth Factor (VEGF) is decreased in the pathogenesis of POEMS syndrome.
Vascular Endothelial Growth Factor (VEGF) is decreased in the pathogenesis of POEMS syndrome.
What is the commonest type of heavy chain disease?
What is the commonest type of heavy chain disease?
The condition characterized by chronic inflammatory demyelinating polyneuropathy is known as __________.
The condition characterized by chronic inflammatory demyelinating polyneuropathy is known as __________.
Match the following heavy chain disease types with their associated features:
Match the following heavy chain disease types with their associated features:
What is the most common cause of sudden cardiac death in patients with amyloidosis?
What is the most common cause of sudden cardiac death in patients with amyloidosis?
Normal serum protein electrophoresis is typically observed in patients with amyloidosis.
Normal serum protein electrophoresis is typically observed in patients with amyloidosis.
What type of biopsy is preferred when investigating amyloidosis?
What type of biopsy is preferred when investigating amyloidosis?
In 2° amyloidosis, patients may present with __________ range proteinuria.
In 2° amyloidosis, patients may present with __________ range proteinuria.
Match the following clinical features with their associated manifestations:
Match the following clinical features with their associated manifestations:
What are non-branching fibrils associated with Primary AL Amyloidosis?
What are non-branching fibrils associated with Primary AL Amyloidosis?
Insoluble amyloid fibrils have a uniform width range of 10-15 nm.
Insoluble amyloid fibrils have a uniform width range of 10-15 nm.
What is the primary role of thrombopoietin in thrombopoiesis?
What is the primary role of thrombopoietin in thrombopoiesis?
What type of amyloidosis is associated with light chain proteins?
What type of amyloidosis is associated with light chain proteins?
Endomitosis is the process of differentiation of megakaryocytes.
Endomitosis is the process of differentiation of megakaryocytes.
The positive reaction of amyloid when stained with congo red is referred to as _______.
The positive reaction of amyloid when stained with congo red is referred to as _______.
Name the type of megakaryocyte associated with immune thrombocytopenic purpura.
Name the type of megakaryocyte associated with immune thrombocytopenic purpura.
Match the soluble precursor with its corresponding type of amyloidosis:
Match the soluble precursor with its corresponding type of amyloidosis:
The process that requires IL-3 for platelet development is known as __________.
The process that requires IL-3 for platelet development is known as __________.
Match the type of megakaryocyte with its associated condition:
Match the type of megakaryocyte with its associated condition:
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Study Notes
Hematology
- Active space manifestations:
- Nocturnal diarrhea
- Gustatory sweating
- Postural hypotension
- Reflex tachycardia
- Carpal tunnel syndrome is common in men.
- Anemia presents as progressive fatigue.
- Hemorrhagic manifestations:
- Macroglossia
- Waxy eyelids and papules
- Poor prognosis factors:
- Elevated NT-Pro BNP
- Elevated serum Trop T
- Decreased free light chains
- Management includes:
- High-dose intravenous melphalan and dexamethasone
- Autologous transplant
Waldenstrom's Macroglobulinemia
- AKA Lymphoplasmacytic Lymphoma
- Pathogenesis:
- Originates from memory B-cells
- Associated with MYD88 and CXCR4 mutations
- Lymphoplasmacytic cells express CD19, CD20, CD38, CD135, and surface IgM
- Clinical Features:
- Peripheral circulation:
- Cold agglutination autoimmune hemolytic anemia
- Hyperviscosity causing headache, tinnitus, and dizziness
- Epistaxis
- Neuropathy
- Raynaud-like symptoms
- Peripheral circulation:
- Diagnosis:
- Criteria:
- IgM monoclonal gammopathy of any concentration
- Small lymphocytic infiltration of bone marrow
- Plasmacytoid/plasma differentiation
- Markers: Surface IgM+, CD5+, CD10+, CD19+, CD20+, CD22, CD23, CD25-, CD27+, FMC 7+, CD103, CD138+
- Investigations:
- Serum protein electrophoresis showing an IgM m-band
- Bone marrow:
- Anemia
- Pancytopenia (rare)
- Weight loss
- Criteria:
Plasma Cell Disorders
-
Cord compression symptoms:
- Compressive neuropathy
- Radiculopathy
- Neuromyeloma (due to vertebral fracture)
-
Causes of neuro-manifestations:
- Hypercalcemia
- Hyperviscosity (due to increased plasma cells)
- Headache, Tinnitus
- Psychiatric overtones
- Carpal tunnel syndrome in males (due to amyloidosis)
- Cord compression symptoms
- Plasma cells
- Plasmacytosis
- Rouleaux formation
-
Poor Prognostic Factors:
- Lambda or free light chains
- IgA, D, E myeloma
- Elevated β2-microglobulin
- Elevated circulating plasma cells
- Decreased serum albumin
- Elevated LDH
-
Management:
- Standard risk:
- Induction: 3 cycles of bortezomib + lenalidomide
- High risk:
- Carfilzomib + lenalidomide + dexamethasone
- If eligible for consolidation:
- Autologous transplant (extract stem cells → ablation → reinfusion → engraftment in 2 weeks)
- Daratumumab (Anti-CD38): Used in the consolidation phase, unfit for transplant, and relapse cases
- Standard risk:
Plasma Cell Disorders (continued)
- Normal serum protein electrophoresis: Shows a smooth, bell-shaped curve.
- Monoclonal m-band: Shows a tall, sharp peak in the middle.
Bone Marrow Biopsy
- Microscopic views: Microscopic image of bone marrow cells present.
- Description (Likely): The image shows numerous cells with dark nuclei, typical of plasma cells.
Serum Markers
- Increased levels of Ca+, BUN, creatinine, uric acid, and β2 microglobulin are observed.
Multiple Myeloma Classifications (MSMART 3.0)
-
High Risk Multiple Myeloma:
- 17p deletion
- t(14; 16)
- t(14; 20)
- t(4; 14)
-
Standard Risk Multiple Myeloma:
- Trisomies
- t(1; 14)
- t(6; 14)
-
Additional Risk Factors:
- Double hit mm: 2+ risk factors
- Triple hit mm: 3+ risk factors
- p53 mutation
- Gain 1q
- β2 microglobulin
Hematology
- Pathogenesis of Multiple Myeloma:
- B-lymphocyte → Primary cytogenetic abnormalities → MGUS → Random second-hit → Multiple myeloma:
- Ras mutation
- c-myc abnormalities
- ↑ IL-6
- Detection of plasma cells > 100 cells/mic.
- Isolated light chain Ig → Nephrotoxic.
- Most common hematological malignancy: Leukemia.
- B-lymphocyte → Primary cytogenetic abnormalities → MGUS → Random second-hit → Multiple myeloma:
- Clinical Presentation:
- Age > 70 years (practical > 40 years)
- Gender: Male > female
- Bone pain and lytic lesions:
- Most common: Back pain
- Increased pain on movement
- Fracture due to trivial trauma
- Cause: Osteoclast activation
- Hypercalcemia
- Nephrogenic DI → Prerenal AKI
- Normal ALP (No bone formation)
- Recurrent Infections: Hypogammaglobulinemia → Pneumonia, UTI.
- Acute Kidney Injury:
- Overflow proteinuria: 24 hours urine protein to identify light chain
- Light chain cast nephropathy (LCCN): Light chains cause interstitial nephritis. λ > κ
- Fractured cast (Not visualized in urine)
- Light chain deposition disease (LCDD): Nodular glomerulosclerosis (κ > λ)
- Proximal renal tubular acidosis (Type 2)
Platelets
- Lifespan of platelets is 10 days.
- Normal count: 1.5 - 4 lakh/μL
- Thrombocytopenia: < 1,00,000/μL
- Bleeding risk: < 20,000/μL
- Significant bleeding risk: < 10,000/μL
- Lifespan in stored blood: 72 hours
- Investigations:
- Coagulation studies: Platelet-poor plasma
- Platelet studies: Platelet-rich plasma
- Conducted at room temperature (exception: Lupus anticoagulant at 4°C)
- Other information:
- AKA satellitism: Caused by EDTA
- Clumping of platelets: Identified by 3.2% trisodium citrate
Platelet Transfusion
- Completion time: Within 20 minutes
Types of Platelet Transfusion
| Type | Process | Cost |
|---|---|---|
| Single donor | Apheresis | Costly |
| Random donor | Pooling & centrifugation | Cheap |
Post-Transfusion Increase in Platelet Count
- SDP: ↑ by 30,000-50,000/μL
- RDP: ↑ by 6,000/μL (4-6 units)
Management
- Plasmapheresis (PLEX)
- BR regimen (4-6 cycles):
- Bendamustine
- Rituximab
POEMS Syndrome
- Pathogenesis: ↑ VEGF (vascular endothelial growth factor)
- Associated with Castleman's Disease
- Major criteria: P & M
- P: Polyneuropathy (CIDP → Chronic inflammatory demyelinating polyneuropathy)
- O: Organomegaly
- E: Edema (extravascular overload)
- M: m-protein
- Minor criteria:
- Endocrinopathy (eg: Hypogonadism)
- S: Sclerotic bone lesions
- Skin changes
Heavy Chain Disease
- Different types based on heavy chains: | Types | Associations and features | |---|---| | a chain disease (m/c) AKA Seligman's disease, immuno-proliferative small intestinal disorder (IPSID) | * Familial Mediterranean lymphoma.* Campylobacter jejuni infection.| | γ chain disease AKA Franklin's disease | * Autoimmune diseases.* Fever + anemia + hepatosplenomegaly.* Palatal edema.| | µ chain disease | * Chronic Lymphocytic Leukemia (CLL).* Vacuoles in lymphocytes.|
Investigations (Amyloidosis)
- Biopsy:
- Abdominal pad of fat (preferred)
- Renal
- Congo red +ve
- Immunohistochemistry:
- +ve for Amyloid A / transthyretin, etc.
- Serum protein electrophoresis: Normal.
- Serum immunoelectrophoresis: ↑λ proliferation (m/c)
Clinical Presentation (Amyloidosis)
- 1. Heart:
- Restrictive cardiomyopathy (RCMP):
- Most common type of cardiomyopathy
- Non ischemic cardiomyopathy
- ECG: Low voltage
- ECHO: Hypertrophy
- Sudden cardiac death (SCD): Most common cause of death
- Restrictive cardiomyopathy (RCMP):
- 2. Kidney:
- In secondary amyloidosis
- Nephrotic range proteinuria with/without nephrotic syndrome (in elderly)
- 3. Liver:
- Hepatomegaly with increased ALP
- Biopsy is contraindicated due to increased risk of bleeding (Anti-xa antibodies)
- 4. Sensory motor axonal neuropathy
- 5. Chronic inflammatory demyelinating polyneuropathy (CIDP)
Platelets - Basics
- Formation of Platelets:
- Common myeloid progenitor → RBC → Granulocyte → Monocyte → Megakaryocyte → Platelet
- Early Thrombopoiesis:
- Requires:
- Thrombopoietin (from liver)
- IL-3 (platelet development)
- IL-II
- Recombinant IL-II (Oprelvekin): Rx of induced thrombocytopenia in chemotherapy
- Requires:
- Late Thrombopoiesis:
- Requires SDF-1
- Stages of Megakaryocyte Development:
- Stage II: Basophilic
- Stage III: maximal granules, multilobed nuclei
- Stage IV: mature, lesser granules, pyknotic nuclei
- Endomitosis: Process of multiplication of megakaryocytes
Morphological Variation of Megakaryocytes
- Giant megakaryocyte: Immune thrombocytopenic purpura
- Dwarf megakaryocyte: Chronic myeloid leukemia
- Cloud megakaryocyte: Primary myelofibrosis
- Staghorn megakaryocyte: Essential thrombocytosis
Primary AL Amyloidosis
- AKA Light chain disease
- Pathogenesis:
- **Soluble Precursors: ** (examples: Serum Amyloid A, light chain, transthyretin, etc.)
- Possibly due to: Misfolding or protein conformation disorder (α → β).
- Insoluble Amyloid:
- Extracellular: Located outside of cells
- Non-branching fibrils: Thread-like structures without branches
- Indefinite length: Variable length
- 7.5-10 nm width: Specific size range
- Congor red (stain) +ve: A positive reaction when stained with congo red.
- **Soluble Precursors: ** (examples: Serum Amyloid A, light chain, transthyretin, etc.)
Classification of Amyloidosis
| Soluble precursor | Type | Clinical Syndrome |
|---|---|---|
| Light chain | AL | Primary amyloidosis |
| Serum amyloid A | AA | Secondary amyloidosis: in kidney (associated with chronic inflammatory condition: TB, rheumatoid arthritis, bronchiectasis etc) |
| ABâ‚‚ microglobulin | ABâ‚‚M | Dialysis associated |
| Transthyretin | ATTR | Senile systemic: wild type, FAP (Familial amyloid polyneuropathy): mutant type |
| Amyloid β protein | AB | Alzheimer's disease, Cerebral amyloid angiopathy |
| Cystatin C | ACYS | |
| Calcitonin | ACal | medullary carcinoma of thyroid |
| Atrial natriuretic factor | AANF | Atrial fibrillation |
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